CURRICULUM VITAE

Richard J. Youle, Ph.D.

National Institutes of Health

Surgical Neurology Branch, NINDS

Bldg. 10 Room 5D37

Bethesda, Maryland 20892

(301) 496-6628

updated 4-11-12

Personal

Born: September 20, 1952

Concord, California

Citizenship: United States Married, four children

Education

June 1974 A.B., Albion College, Albion, Michigan

December 1977 Ph.D., University of South Carolina, Columbia,

South Carolina

Professional Experience

1974 - 1976 Teaching Fellow, University of South Carolina, Columbia,

South Carolina

1976 - 1977 Research Assistant, Dr. Anthony Huang, University of South

Carolina, Columbia, South Carolina

1977 - 1981 Staff Fellow, Laboratory of Neurochemistry, NIMH,

Bethesda, Maryland

1981 - 1982 Senior Staff Fellow, Laboratory of Neurochemistry, NIMH,

Bethesda, Maryland

1982 - 1984 Research Chemist, Laboratory of Neurochemistry, NIMH, Bethesda

1984 - 1988 Research Chemist, Surgical Neurology Branch, NINDS, Bethesda

1988 - Present Chief, Biochemistry Section, Surgical Neurology Branch, NINDS

Awards

1988 Pierce Immunotoxin Award

1992 NIH Directors Award

1998 Mentor Award, Inventor's Hall of Fame

2011 NIH Directors Award

CRADAs

1987-1996 "Development of Immunotoxins for CNS Disease" Hafslund Nycomed

1988-1989 "Angiogenin Immunotoxins" Biogen Corporation

1993 -1999 "Mechanism of Cytotoxic RNases" Alfacell Inc.

1996 -2000 "Development of Improved Immunotoxins" Nycomed Imaging

2002-2011 “Immunotoxins for Brain tumors” KS Biomedix

Editorial and Committee Duties

1987 - 1994 NINDS Tenure and Promotion Committee

1991 Consultant for NIMH Scientific Counselors, Child Psychiatry Branch

1991 Site Reviewer for an NCI program project grant (PO1) to the

University of Minnesota

1994 Consultant, Dutch Cancer Society

1995 Cancer Therapy Study Section, Ad hoc member

1995 Immunology Study Section, Ad hoc member

1995, 1996 Thesis Examiner, University of Delhi

1991 - Editorial Board of Drug Targeting and Delivery

1992 -2000 NINDS Technology Transfer Advisory Committee

1996 -1999 Consultant in Research, George Washington University

1996 -1999 NIMH Tenure and Promotion Committee

1996 -1999 Chairman, Organizing Committee for the 5th International Meeting

on Ribonucleases

1996 - NINDS Tenure and Promotion Committee

1997 - Steering Committee, NIH Apoptosis Interest Group

1998 Reviewer for “Comitato Promotore Telethon” and the Neuromuscular

Disease Research Fund

1998 Consultant for the “Italian Association of Cancer Research”

1998 Ad hoc member of “Brain Disorders and Clinical Neurosciences-4

1998 Study Section, " Brain Disorders and Clinical Neurosciences-3

II Study Section”

1999 MIPS (Maryland Industrial Partnerships) technical reviewer

1999-2003 Ph.D. Thesis Committee, Johns Hopkins University

2000 Brain Tumor Progress Review Group, Leesburg, VA

2000, 2002, 2009 The Wellcome Trust, grant reviewer

2001 -2009 Member, Faculty of 1000, web-based research synopsis

2001 Italian Association for Cancer Research, grant proposal review

2001-2004 Co-chairman, NINDS Technology Advisory Committee

2001-2006 Study Section of the American Cancer Society: Cell

Cycle and Growth Control

2001- Head, Apoptosis Interest Group of NIH

2002- Member, NIH CRADA subcommittee

2002- NIMH Tenure and Promotion Committee

2003 Ad hoc member, Special Toxin Study Section, NIGMS

2003 Neuro-Oncology Branch, NCI, tenure–track search committee

2003 Ad hoc Reviewer for the Marsden Fund of the Royal Society of New Zealand

2003 Reviewer for the Science Foundation of Ireland

2004- Oxford/Cambridge/NIH GPP, Graduate Student Director 2004 class

2004 Program Project Site Reviewer for the National Cancer Institute of Canada,

University of Toronto, Toronto

2004-2008 External Advisor, PO1 Program Project Grant to the Medical University of South

Carolina, Charleston

2006-2009 Editorial Board, Cell Death and Differentiation

2006- NINDS Tenure and Promotion Committee

2007 Ad hoc member, Finnish Academy of Sciences grant review panel, Helsinki

2007 US-Israel Binational Science Foundation, Jerusalem

2007-2009 Adjunct Professor, George Washington University

2008 Association for International Cancer Research, UK

2007 NCI Search Committee, Laboratory of Molecular Biology

2007 NINDS Neurosurgery Branch Search Committee

2007 NINDS, Cell and Membrane Signaling Group Search committee

2008 Cancer Research Campaign UK grant reviewer

2008, 2009 MRC grant reviewer

2008 Co-Chair, Gordon Conference on Cell Death, Montana

2008-2009 Scientific Advisory Board, Target-In Inc., Israel

2009-2010 Clinical Associate Professor, George Washington University

2009-2012 Editorial Board, Current Opinion in Cell Biology

2009- Editorial Board, Journal of Cell Biology

2009- MEXT Japanese Program Project Advisor, Department of Physiology and

Cell Biology, Tokyo Medical and Dental University

2009-2010 Ph.D. Thesis Committee, Rockefeller University

2011 Organizing Committee, Mitochondrial Biology in Cardiovascular Health and

Diseases Conference, NIH

2011 Chair, NICHD search committee, CBMP tenure track investigator

2010- Editorial Board, Autophagy

2012- Board of Reviewing Editors, eLife

Patents

U.S. #4,359,457 Anti-Thy 1.2 Monoclonal Antibody-Ricin Hybrid utilized as a tumor suppressant.

U.S. #4,356,117 Chemical modifications of proteins which induce new receptor specificities and therefore elicit new effects in cells.

U.S. #4,397,843 Mannose-6-phosphate-low density protein reagent effective against hypercholesterolemia.

U.S. #4,440,747 Monoclonal antibody-ricin hybrids as a treatment of murine graft-versus host disease. U.S. #4,500,637 Prevention of graft-versus-host disease following bone marrow transplantation.

U.S. #4,520,011 Inactivating protein synthesis by incubating anti-thy 1.1-ricin A chain monoclonal antibody

hybrids with target protein cells.

U.S. #4,520,226 Treatment of graft-versus-host disease using a mixture of T-lymphocyte specific monoclonal antibody: ricin conjugates. U.S.

U.S. #5,208,021 Method of preparing diphtheria immunotoxins.

Licensed to Nycomed Amersham Licensed to Novartis

U.S. #5,352,447 Immunotoxins for the treatment of intracranial lesions and as adjunct to chemotherapy..

Licensed to Nycomed Amersham U.S. # 5,728,383 Treatment of Tumors of the Central Nervous System with Immunotoxins.

Licensed to Nycomed Amersham

Licensed to Novartis U.S. # 5,792,458 Mutant Diphtheria Toxin Conjugates.

Licensed to Nycomed Amersham

Licensed to Novartis U.S. #5,840,840 Selective RNase Cytotoxic Reagents

Licensed to Antisome Research Ltd. U.S. #5,955.073 Selective RNase Cytotoxic Reagents

Licensed to Antisome Research Ltd. U.S. #6,696,064 Methods of Protecting Vasculature from Damage by Diphtheria toxin-and Pseudomonas

toxin-based Immunotoxins During Therapy

Licensed to KS Avicenna

WO2010/0317577 Methods and compositions for inhibiting cell death or enhancing cell proliferation

Licensed to Protox

WO2011/053825 Compositions and methods for the treatment or prevention of mitochondrial diseases

Peer Reviewed Papers

1. Youle RJ and Huang AHC: Development and properties of fructose 1,6-bisphosphatase in the

endosperm of castor-bean seedlings. Biochem J 154:647-652, 1976.

2. Huang, AHC, Liu KDF and Youle RJ: Organelle-specific isozymes of asparate-o-ketoglutarate

transaminase in spinach leaves. Plant Physiol 58:110-113, 1976.

3. Youle RJ and Huang AHC: Protein bodies from the endosperm of castor bean: Subfractionation,

protein components, lectins, and changes during germination. Plant Physiol 58:703-710, 1976.

4. Youle RJ and Huang AHC: The albumin and globulin storage proteins in the protein bodies of

castor bean. Plant Physiol 61:13-18, 1978.

5. Youle RJ and Huang AHC: Evidence that the castor bean allergens are the albumin storage

proteins in the protein bodies of castor bean. Plant Physiol 61:1040-1042, 1978.

6. Youle RJ, Murray GJ and Neville DM, Jr.: Ricin linked to monophosphopentamannose binds to

fibroblast lysosomal hydrolase receptors results in a cell type-specific toxin.

Proc Natl Acad Sci, USA 76:5559-5562, 1979.

7. Youle RJ and Huang AHC: Albumin storage protein and allergens in cottonseeds.

J Agric Food Chem 27:500-503, 1979.

8. Youle RJ and Neville DM: Receptor-mediated transport of the hybrid protein ricin-diphtheria

toxin fragment A with subsequent ADP-ribosylation of intracellular elongation factor-II.

J Biol Chem 254:11089-11096, 1979.

9. Youle RJ and Neville DM: Anti-Thy 1.2 monoclonal antibody linked to ricin is a potent cell-type-

specific toxin. Proc Natl Acad Sci, USA 77:5483-5486, 1980.

10. Youle RJ and Huang AHC: Occurrence of low molecular weight and high cysteine containing

albumin storage proteins in oilseed of diverse species.

Am J Botany 68:44-48, 1981.

11. Robbins AR, Myerowitz R, Youle RJ, Murray GJ and Neville DM, Jr.: The mannose 6-phosphate

receptor of Chinese hamster ovary cells. Isolation of mutants with altered receptors. J Biol Chem

256:10618-10622, 1981.

12. Youle RJ, Murray GJ and Neville DM Jr.: Studies on the galactose binding site of ricin and the

hybrid toxin man 6P-ricin. Cell 23:551-559, 1981.

13. Vallera DA, Youle RJ, Neville DM, Jr. and Kersey JH: Bone marrow transplantation across major

histocompatability barriers. III. Protection of mice from lethal GVHD by pretreatment of donor

cells with monoclonal anti-Thy 1.2 coupled to the toxin ricin. J Exp Med 155:949-954, 1982.

14. Youle RJ and Neville DM, Jr.: Kinetics of protein synthesis inactivation by ricin-anti-thy 1.1

monoclonal antibody hybrids: Role of the ricin B subunit demonstrated by reconstitution.

J Biol Chem 257:1598-1601, 1982.

15. Vallera DA, Youle RJ, Neville DM, Jr., Soderling CB and Kersey JH: Bone marrow

transplantation across major histocompatibility barriers in mice. VI. Anti T-cell monoclonal

antibody-toxin conjugates as reagents for experimental GVHD prophylaxis are not reactive with

murine stem cells. Transplantation 36:73-79, 1983.

16. Vallera DA, Ash RC, Zanjani ED, Kersey JH, LeBien TW, Beverley PCL, Neville DM, Jr. and

Youle RJ: Anti-T-cell reagents for human bone marrow transplantation: Ricin linked to three

monoclonal antibodies. Science 222:512-515, 1983.

17. Quinones RR, Youle RJ, Kersey JH, Azajani ED, Azemove SM, Soderling CCB, LeBien TW,

Beverley PCL, Neville DM, Jr., and Vallera DL: Anti-T cell monoclonal antibodies conjugated to

ricin as potent reagents for GVHD profilaxis: Effect on the generation of cytotoxic T cells in both

peripheral blood and bone marrow.

J Immunol 132:678-683, 1984.

18. Filipovich MD, Vallera DA, Youle RJ, Quinones RA, Neville DM, Jr. and Kersey JH: Ex vivo

treatment of donor bone marrow with anti T-cell immunotoxins for the prevention of graft vs. host

disease. Lancet 1:469-472, 1984.

19. Stong R, Youle RJ and Vallera DA: Elimination of clonogenic T-leukemic cells from human bone

marrow using anti-Mr 65,000 protein immunotoxins. Cancer Research 44:3000-3006, 1984.

20. Uckun FM, Stong RC, Youle RJ and Vallera DA: Combined ex vivo treatment with immunotoxins

and mafosfamid: A novel immuno-chemotherapeutic approach for elimination of neoplastic T-cells

from autologous marrow grafts. J Immunol 134:3504-3515, 1985.

21. Murray GJ, Youle RJ, Gandy SE, Zirzow GC and Barranger J: Purification of B-

glucocerebrosidase by preparative scale HPLC: The use of ethylene glycol containing buffers for

chromatography of hydrophobic glycoprotein enzymes. Anal Biochem 147:301-310, 1985.

22. Stong RC, Uckun FM, Youle RJ, Kersey JH and Vallera DA: Use of multiple T cell directed intact

ricin immunotoxins for autologous bone marrow transplantation.

Blood 66:627-635, 1986.

23. Youle RJ, Uckun FM, Vallera DA and Colombatti M: Immunotoxins show rapid entry of

diphtheria toxin but not ricin via the T3 antigen. J Immunol 136:93-98, 1986.

24. Colombatti M, Greenfield L and Youle RJ: Cloned fragment of diphtheria toxin linked to T cell

specific antibody identifies region of B chain active in cell entry.

J Biol Chem 261:3030-3035, 1986.

25. Lewis MS and Youle RJ: Ricin subunit association: Thermodynamics and the role of the disulfide

bond in toxicity. J Biol Chem 261:11571-11577, 1986.

26. Zovickian J, Johnson VG and Youle RJ: Potent and specific killing of human malignant brain

tumor cells by an anti-transferrin receptor antibody-ricin immunotoxin.

J Neurosurg 66:850-861, 1987.

27. Colombatti M, Johnson VG, Skopicki HA, Fendley B, Lewis MS and Youle RJ: Identification and

characterization of a monoclonal antibody recognizing a galactose-binding domain of the toxin

ricin. J Immunol 138:3339-3344, 1987.

28. Youle RJ and Colombatti M: Hybridoma cells containing intracellular anti-ricin antibodies show

ricin meets secretory antibody before entering the cytosol.

J Biol Chem 262:4676-4682, 1987.

29. Gregg RO, Bridges SA, Youle RJ, Longo DL, Houston LL, Glennie MJ, Stevenson FD and Green

I: Whole ricin and recombinant ricin A chain idiotype-specific immunotoxins for therapy of the

guinea pig L2C B cell leukemia. J Immunol 138:4502-4508, 1987.

30. Filipovich AH, Vallera DA, Youle RJ, Haake R, Blazar B, Arthur D, Neville DM, Ramsay N,

McGlave P and Kersey JH: Graft versus host disease prevention in allogeneic bone marrow

transplantation: A pilot study using immunotoxins for T cell depletion of donor bone marrow.

Transplantation 44:62-69, 1987.

31. Greenfield L. Johnson VG and Youle RJ: Point mutations in diphtheria toxin separate binding

from entry and amplify immunotoxin selectivity. Science 238:536-539, 1987.

32. Johnson VG, Wilson D, Greenfield L and Youle RJ: The role of the diphtheria toxin receptor in

cytosol translocation. J Biol Chem 263:1295-1300, 1988.

33. Boltansky H, Slater J, Youle RJ, Isersky C and Kaliner M: IgE immunotoxins II. IgE-Ricin A

chain. Immunopharmacology 14:47-62, 1988.

34. Zovickian J and Youle RJ: Intrathecal immunotoxin therapy in an animal model of leptomeningeal

neoplasia. J Neurosurg 68:767-774, 1988.

35. Johnson VG and Youle RJ: A point mutation of proline 308 in diphtheria toxin B chain inhibits

membrane translocation of toxin conjugates. J Biol Chem 264:17739-17744, 1989.

36. Johnson VG, Wrobel C, Wilson D, Zovickian J, Greenfield L, Oldfield EH, and Youle RJ:

Improved tumor-specific immunotoxins in the treatment of CNS and leptomeningeal neoplasia. J

Neurosurg 70:240-248, 1989.

37. Sung C, Youle RJ, and Dedrick RL: Pharmacokinetic Analysis of Immunotoxin Uptake in Solid

Tumors: Role of Plasma Kinetics, Capilary Permeability and Binding.

Cancer Research 50:7382-7392, 1990.

38. Hall WH, Merrill MJ, Walbridge S and Youle RJ: Epidermal growth factor receptors on

ependymomas and other brain tumors. J Neurosurg 72:641-646, 1990.

39. Wrobel CW, Wright DC, Dedrick RL and Youle RJ: Intravenous Diphtheria Toxin Extends

Survival in Nude Rats Bearing an Intracranial Human Tumor Zenograft.

J Neurosurg 72:946-950, 1990.

40. Riedel CJ, Muraszko KM and Youle RJ: Diphtheria Toxin Mutant Selectively Kills Cerebellar

Purkinje Neurons. Proc Natl Acad Sci, USA 87:5051-5055, 1990.

41. Dipasquale B, and Youle RJ: Programmed Cell Death and the Immune System:

Physiopathological Implications of Apoptosis. Editorial, J Immunol Res 3:149-154, 1991.

42. Jin FS, Youle RJ, Johnson VG, Shiloach J, Fass R, Longo DL, and Bridges, SH: Suppression of the

Immune Response to Immunotoxins with Anti-CD4 Monoclonal Antibodies. J Immunol

146:1806-1811, 1991.

43. Sung C, Wilson D, and Youle RJ: Comparison of Protein Synthesis Inhibition Kinetics and Cell

Killing Induced by Immunotoxins. J Biol Chem 266:14159-14162, 1991.

44. Rybak SM, Saxena S, Ackerman E and Youle RJ: Cytotoxic Potential of Ribonuclease and

Ribonuclease Hybrid Proteins. J Biol Chem 266:21202-21207, 1991.

45. Saxena S, Rybak SM, Winkler G, Meade HM, Youle RJ, and Ackerman E: Comparison of RNases

and Toxins Upon Injection into Xenopus Oocytes.

J Biol Chem 266:21208-21214, 1991.

46. Dipasquale B, Marini AM and Youle RJ: Apoptosis and DNA Degradation Induced by 1-Methyl-

4-Phenylpyridinium (MPP+) in Neurons.

Biochem Biophys Res Comm 181:1442-1448, 1991.

47. Rybak SM, Hoogenboom HR, Meade HM, Raus JCM, Schwartz D and Youle RJ: Humanization

of Immunotoxins. Proc Natl Acad Sci, USA 89:3165-3169, 1992.

48. Newton DL, Wales R, Richardson PT, Walbridge S, Saxena SK, Ackerman EJ, Roberts LM, Lord

JM and Youle RJ. Cell Surface and Intracellular Functions for Ricin Galactose Binding. J Biol

Chem 267:11917-11922, 1992.

49. Newton DL, Ilercil O, Laske DW, Oldfield EH, Rybak SR, and Youle RJ: Cytotoxic Ribonuclease

Chimeras: Targeted Tumoricidal Activity In Vitro and In Vivo.

J Biol Chem 267:19572-19578, 1992.

50. Saxena SK, Rybak SM, Davey RT, Youle RJ, and Ackerman EJ: Angiogenin Is a Cytotoxic,

tRNA-specific Ribonuclease in the RNase A Superfamily.

J Biol Chem 267:21982-21986, 1992.

51. Dipasquale B and Youle RJ: Programmed Cell Death in Heterokaryons: A Study of the Transfer

of Apoptosis Between Nuclei. Am J Pathology 141:1471-1479, 1992.

52. Johnson VG, Nicholls PJ, Habig WH, and Youle RJ: The Role of Proline 345 in Diphtheria Toxin

Translocation. J Biol Chem 268:3514-3519, 1993.

53. Nicholls PJ, Johnson VG, Andrew SM, Hoogenboom HR, Raus JC and Youle RJ:

Characterization of Single-Chain Antibody (sFv)-Toxin Fusion Proteins Produced In Vitro in

Rabbit Reticulocyte Lysate. J Biol Chem 268:5302-5308, 1993.

54. Wu Y-N., Mikulski S.M., Ardelt W, Rybak SM, and Youle RJ: A Cytotoxic Ribonuclease. Study

of the Mechanism of Onconase Cytotoxicity. J Biol Chem 268:10686-10693, 1993.

55. Sung C, Dedrick RL, Hall WA, Johnson PA and Youle RJ: The Spatial Distribution of

Immunotoxins in Solid Tumors: Assessment by Quantitative Autoradiography.

Cancer Research 53:2092-2099, 1993.

56. Muraszko K, Sung C, Walbridge S, Greenfield L, Dedrick R, Oldfield EH and Youle RJ:

Pharmacokinetics and Toxicology of Immunotoxins Administered into the Subarachnoid Space in

Nonhuman Primates and Rodents.

Cancer Research 53:3752-3757, 1993.

57. Wood KA, Dipasquale B and Youle RJ: In situ Labeling of Granule Cells for DNA

Fragmentation Associated with Apoptosis Shows Different Mechanisms of Cell Loss in the

Developing Cerebellum. Neuron 11:621-632, 1993.

58. Laske DW, Illercil O, Akbasak A, Youle RJ and Oldfield EH: Efficacy of Direct Intratumoral

Therapy with Targeted Protein Toxins for Solid Human Gliomas in Nude Mice. J Neurosurg

80:520-526, 1994.

59. Newton DL, Walbridge S, Ardelt W, Mikulski SM, Rybak SM and Youle RJ: Onconase,

Eosinophil-Derived Neurotoxin and RNase A Neurotoxicity to Purkinje Cells.

J Neurosci 14:538-544, 1994.

60. Wu Y-N, Gadina M, Chang S and Youle RJ: Retinoic Acid Disrupts the Golgi Apparatus and

Potentiates Select Immunotoxins. J Cell Biol 125:743-753, 1994.

61. Youle RJ, Wu Y-N, Mikulski M, Shogen K, Hamilton RS, Newton D, D'Alessio G and Gravell M:

RNase Inhibition of HIV Infection of H9 cells.

Proc Natl Acad Sci, USA 91:6012-6016, 1994.

62. Newton DL, Nicholls PJ, Rybak SM. and Youle RJ: Expression and Characterization of

Recombinant Human Eosinophil-Derived Neurotoxin and Eosinophil-Derived Neurotoxin-anti-

transferrin Receptor sFv. J Biol Chem 269:26739-26745, 1994.

63. Lin JJ, Newton DL, Mikulski SM, Kung HF, Youle RJ and SM Rybak: Anti-Tumor Protein

Targets tRNA in Rabbit Reticulocyte Lysates to Cause Protein Synthesis Inhibition. Biochem

Biophys Res Comm 294:156-162, 1994.

64. Wu Y-N, Saxena SK., Ardelt W, Mikulski SM, DeLorenzo C, D'Alessio G and Youle RJ: A study

of the Intracellular Routing of Cytotoxic Ribonucleases.

J Biol Chem 270:17476-17481, 1995.

65. Wood KA and Youle RJ: The Role of Free Radicals and p53 in Neuron Apoptosis In Vivo. J

Neurosci 15:5851-5856, 1995.

66. Boix E, Wu Y-N, Saxena SK, Vasandani V, Ardelt W, Ladner J and Youle RJ: Role of the N-

terminus in RNase A homologues: differences in catalytic activity, ribonuclease inhibitor

interaction and cytotoxicity. J Mol Biol 257:992-1007, 1996.

67. Mosimann SC, Newton DL, Youle RJ, and James MNG: X-ray Crystallographic Structure of

Recombinant Eosinophil-Derived Neurotoxin at 1.83A Resolution.

J Mol Biol 260:540-552, 1996.

68. Saxena SK, Gravell M, Wu Y-N, Mikulski SM, Shogen K, Ardelt W and Youle RJ: Inhibition of

HIV-1 Production and Selective Degradation of Viral RNA by an Amphibian Ribonuclease. J Biol

Chem 271:20783-20788, 1996.

69. Vasandani VM, Wu Y-N, Mikulski SM, Youle RJ and Sung C: Molecular Determinants in the

Plasma Clearance and Tissue Distribution of Ribonucleases of the RNase Superfamily. Cancer

Research 56:4180-4186, 1996.

70. Hsu, Y-T, Wolter, K, and Youle RJ: Cytosol to Membrane Redistribution of Members of the Bcl-2

Family During Apoptosis. Proc Natl Acad Sci 94:3668-3672, 1997.

71. Hsu, Y-T and Youle RJ: Nonionic detergent induced dimerization of members of the Bcl-2 family.

J Biol Chem 272: 13829-13834, 1997.

72. Laske, DW, Muraszko. K, Oldfield, EH, DeVroom, H, Sung, C, Dedrick, R, Simon, T,

Colendra, C, Katz, D, Groves, E, Greenfield, L, Houston, L, and Youle RJ: Intraventricular

Immunotoxin Therapy for Leptomeningeal Neoplasia.

Neurosurgery 41: 1039-1049, 1997.

73. Castelli, JC, Hassel, BA, Wood, KA, Li, XL, Amemiya, K, Dalakas, MC, Torrence, PF and Youle

RJ: A study of the interferon antiviral mechanism: Apoptosis activation by the 2-5A system. J.

Exp. Med. 186: 967-972, 1997.

74. Laske, DW, Youle RJ, and Oldfield, EH: Tumor regression with regional distribution of the

targeted toxin, Tf-CRM107 in patients with malignant brain tumors. Nature Medicine 3: 1362-

1368, 1997.

75. Wolter, KG, Hsu, Y-T, Smith CL, Nechushtan, A, Xi, X-G, and Youle RJ: Movement of Bax from

the cytosol to mitochondria during apoptosis.

J. Cell Biol. 139: 1281-1292, 1997.

76. Hott, JS, Dalakas, MC, Sung, C, Hallett, M and Youle RJ: A skeletal muscle-specific

immunotoxin for the treatment of focal muscle spasm. Neurology 50: 485-491, 1998.

77. Castelli, JC, Hassell, BA, Maran, A, Paranjape, J, Hewitt, JA, Li, X-l, Hsu, Y-T, Silverman, RH

and Youle RJ: The role of 2’-5’ oligoadenylate-activated ribonuclease L in apoptosis. Cell Death

and Differentiation, 5: 313-320, 1998.

78. Hsu, Y-T and Youle RJ: Bax in murine thymus is a soluble monomeric protein which displays

differential detergent-induced conformations. J. Biol. Chem. 273: 10777-10783, 1998.

79. Vasandani, V., Castelli, J., Hott, J., and Youle RJ: Interferon enhances the activity of the

anticancer ribonuclease onconase. Journal of Interferon and Cytokine Research,

19: 447-454, 1999

80. Suzuki, M, Saxena, S.K., Boix, E., Prill, R.J., Vasandani, V.M. , Ladner, J.E., Sung, C.

and Youle RJ: Engineering receptor-mediated cytotoxicity into human ribonucleases by

steric blockade of inhibitor interaction, Nature Biotechnology, 17: 265-269, 1999.

81. Basañez, A. Nechushtan, O. Drozhinin, A. Chanturiya, E. Choe, S. Tutt, K.

Wood, Y.-T. Hsu, J. Zimmerberg, Youle RJ. Full length Bax disrupts

planar phospholipid membranes. Proc. Natl. Acad. Sci. USA, 96: 5492-5497, 1999.

82. Nechushtan, A, Smith, CL, Hsu, Y-T, Youle RJ. Conformation of the Bax C-terminus

regulates subcellular location and cell death. EMBO J. 18: 2330-2341, 1999.

83. Liu, XH, Castelli, JC, and Youle RJ. Receptor mediated uptake of a Bcl-XL fusion protein

inhibits apoptosis. Proc. Natl. Acad. Sci. USA 96: 9563-9567, 1999.

84. Gorbatyuk VY, Chen YC, Wu YJ, Youle RJ, Huang TH. Sequence-specific H-1, C-13 and N-

15 resonance assignments of recombinant onconase/P-30 protein. J. Biomol. NMR. 15: 343-

344, 1999

85. Hagihara, N, Walbridge, S, Olson, AW, Oldfield, EH and Youle RJ. Vascular Protection by

Chloroquine During Brain Tumor Therapy with Tf-CRM107. Cancer Res. 60: 230-234, 2000.

87. Christiansen, SP, Sandnas, A, Prill, R and Youle, RJ and McLoon, LK. Acute Effects of the

Skeletal Muscle-Specific Immunotoxin, Ricin-Mab 35, on Extraocular Muscles of Rabbits.

Investigative Opthamology, 41: 3402-3409, 2000.

88. Ghatan, S, Larner, S, Kinoshita, Y, Hetman, M, Patel, L, Xia, Z, Youle, RJ and Morrison, RS.

P38-Mitogen activated protein kinase mediates Bax translocation and caspase induction in nitric

oxide-induced apoptosis in neurons. J. Cell Biol. 150:335-348, 2000.

89. Suzuki, M, Youle, RJ and Tjandra, N, Structure of Bax: Co-regulation of dimer formation and

intracellular localization. Cell 103: 645-654, 2000.

90. Leonidas, D, Boix, E, Prill, R, Suzuki, M, Turton, R, Minson, K, Swaminathan, Youle, RJ and

Acharya, KR: Mapping the Ribonucleolytic Active Site of Eosinophil Derived Neurotoxin

(EDN): High Resolution Crystal Structures of EDN Complexes with Adenylic Mononucleotide

Inhibitors. J. Biol. Chem. 276: 15009-15017, 2001.

91. Nechushtan, A, Smith,

,CL, Lamensdorf,

I, Yoon

, SH, and Youle

RJ: Bax and Bak

coalesce into novel mitochondria-associated clusters during apoptosis.

J. Cell Biol. 153: 1265-1276, 2001.

92. Smaili, SS, Hsu, YT, Sanders, KM, Russell, JT and Youle, RJ. Bax translocation to

mitochondria subsequent to a rapid loss of mitochondrial membrane potential. Cell Death

and Differentiation. 8:909-920, 2001.

93. Mir SS, Richter BWM, Eiben LJ, Lewis J, Frattini A, Tian L, Frank S,

Youle RJ, Nelson DL, Notarangelo LD, Vezzoni P, Earnhead HO, and Duckett

CS. ILP-2, a novel inhibitor of apoptosis protein that specifically targets

the Apaf-1: Caspase-9 holoenzyme. Mol. Cell Biol 21:4292-4301, 2001.

94. Frank, S, Gaume, B, Bergmann-Leitner ES, Leitner, WW, Robert, EG, Smith, CL and Youle,

RJ. Role of dynamin-related protein 1, a mediator of mitochondrial fission, in apoptosis.

Dev. Cell 1: 515-525, 2001. Commentaries in J. Cell Biol. and Nature Cell Biol.

95. Liu, XH, Collier, RJ and Youle, RJ. Inhibition of axotomy-induced neuronal apoptosis by

extracellular delivery of a Bcl-XL fusion protein. J. Biol. Chem. 276:46326-32, 2001.

96. Christiansen, S.P., Peterson, D., To, T., Youle, RJ., and McLoon, L. Long-term Effects of

Ricin-mAb 35 on Extraocular Muscles of Rabbits: Potential Treatment for Strabismus.

Investigative Ophthalmology and Visual Science. 43: 679-685, 2002.

97. Ichinose, M, Liu, XH, Hagihara, N. and Youle, RJ. Extracellular Bad fused to toxin

transport domains induces apoptosis. Cancer Res. 62, 1433-1438, 2002.

98. F. De Giorgi, L. Lartigue, M. Bauer, A. Schubert, S. Grimm, G. Hanson, S.

Remington, R. J. Youle and F. Ichas. The permeability transition pore signals apoptosis

by directing Bax translocation and multimerization. FASEB J. 16: 607-609, 2002

99. S. K. Saxena, R. Sirdeshmukh, W. Ardelt, S. M. Mikulski, K. Shogen and Youle, RJ. Entry

into cells and selective degradation of tRNAs by a cytotoxic member of the RNase A family.

J. Biol. Chem. 277: 15142-15146, 2002.

100. M. Karbowski, Y-J. Lee, B. Gaume, S.Y. Jeong, S. Frank, A. Nechushtan, A. Santel, M. Fuller,

C.L. Smith, Youle, RJ. Spatial and temporal association of Bax with mitochondrial fission

sites, Drp1, and Mfn2 during apoptosis, J. Cell Biol. 159, 931-938, 2002.

101. Hsu, Y.T., Hou, Q., Cymbalyuk, E. and Youle, RJ. Generation and Characterization of

Species-Specific Anti-Bcl-XL Monoclonal Antibodies. Hybridoma. 22: 91-95, 2003.

102. Putcha, G.V., Le, S., Frank, S., Chu, B., Clark, K., Youle, R.J., LaMarche, A., Maroney, A.C.,

and Johnson, E.M. JNK-mediated BIM phosphorylation potentiates Bax-dependent Apoptosis.

Neuron 38: 899-914, 2003.

103. Santel, A., Frank, S., Gaume, B., Herrler, M., Youle, R.J., and Fuller, M.T. Mitofusin-1

protein is a broadly expressed mediator of mitochondrial fusion in mammalian cells. J. Cell

Sci. 116: 2763-74, 2003.

104. Suzuki, M., Jeong S.Y., Karbowski, M., Youle, R.J. and Tjandra, N., Solution Structure of

Human Mitochondria Fission Protein Fis1 Reveals a Novel TPR-Like helix Bundle. Submitted

to J. Mol. Biol. 334: 445-458, 2003.

105. Arnoult, D., Gaume, B., Karbowski, M., Cecconi, F., and Youle, R.J. Mitochondrial release

of AIF and EndoG requires caspase activation downstream of Bax/Bak-mediated

permeabilization. EMBO J. 22: 4385-4399, 2003.

106. Rostovseva, T.K., Antonsson, B., Suzuki, M., Youle, R.J., Colombini, M., and Bezrukov, S.M.

Bid But Not Bax Regulates the VDAC Channels. J. Biol. Chem 279: 13575-13583, 2004.

107. Karbowski, M., Arnoult, D., Chen, H., Chan D.C., Smith, C., and Youle, RJ.

Quantification of mitochondrial dynamics by photolabeling of individual organelles

shows mitochondrial fusion is blocked during apoptosis upstream of Bax/Bak mediated

caspase activation. J. Cell Biol.164: 493-499, 2004. Commentary in J. Cell Biol.

108. Hofmann, S.R., Lam, A.Q., Frank, S., Zhou, Y-J., Ramos, H.L., Agnello, D., Youle, R.J.,

O’Shea, J. Jak3-independent trafficking of the common gamma chain receptor subunit-

chaperone function of Jaks revisited. Mol. Cell. Biol. 24: 5039-5049, 2004.

109. Jeong, S-Y., Gaume, B., Lee, Y.J., Hsu, Y.T., Ryu, S.W., Yoon, S.H. and Youle, R.J.

Bcl-XL sequesters its C-terminal membrane anchor in soluble, cytosolic homodimers.

EMBO J. 23: 2146-2155, 2004.

110. Arnoult, D., Bartle, L.M., Skaletskaya, A., Zamzami, N., Poncet, D., Park, P., Sharpe, J.,

Youle, R.J., Goldmacher, V.S. Cytomegalovirus cell death suppressor vMIA interacts

with Bax and blocks Bax-mediated apoptosis, but fails to suppress Bak-mediated

apoptosis. Proc. Natl. Acad. Sci. USA 101: 7988-7993, 2004.

111. Szabadkai, G., Simoni, A.M., Wieckowski, M., Youle, R.J. and Rizzuto, R. Drp-1

dependent division of the mitochondrial network blocks intraorganellar Ca2+ waves and

protects against Ca2+ mediated apoptosis. Mol. Cell 8:59-68, 2004.

112. Karbowski, M., Jeong, S-Y. and Youle R.J. Endophilin B1 participates in the

mitochondrial division machinery. J. Cell Biol. 166: 1027-1039, 2004.

113. Lee, Y.J., Jeong, S.J., Karbowski, M., Smith, C.L. and Youle R. J. Roles of the

mitochondrial fission and fusion mediators, Fis1, Drp1 and Opa1, in apoptosis. Mol.

Biol. Cell 15:5001-5011, 2004.

114. Carvalho AC, Sharpe J, Rosenstock TR, Teles AF, Kowaltowski AJ, Youle RJ, Smaili

SS. Bax affects intracellular Ca(2+) stores and induces Ca(2+) wave propagation.

Cell Death Differ. 11: 1265-1276, 2004

115. Nesic-Taylor, O. D. Cittelly, Z. Ye, G-Y. Xu, G. Unabia, J.C. Lee, N.M. Svrakic, X-H

Liu, R.J. Youle, T.G. Wood, D. McAdoo, K.N. Westlund, C.E.Hulsebosch, Jose Regino

Perez-Polo, Exogenous BCL-X(L) Fusion Protein Spares Neurons After Spinal Cord

Injury. J. Neuro. Res. 79: 628-637, 2005.

116. Antignani, A. and R.J. Youle. A chimeric protein induces tumor cell apoptosis by

delivery of the human Bcl-2 family BH-3-only protein Bad. Biochemistry

44: 4074-4082, 2005.

117. Suzuki, M., Neutzner, A., Tjandra, N., and R. J. Youle, Novel structure of the N-

terminus in yeast Fis1 correlates with a specialized function in mitochondrial fission. J.

Biol. Chem. 280: 21444-21452, 2005.

118. Neutzner, A., and R.J. Youle. Instability of the mitofusin Fzo1 regulates mitochondrial

morphology during the mating response in the yeast Saccharomyces cerevisiae. J. Biol.

Chem. 280: 185980-18603, 2005.

119. Takahashi, Y., Karbowski, M., Yamaguchi, H., Kazi, A., Wu, J., Sebti, S.M., Youle, R.J.,

Wang, H.G. Loss of Bif-1 Suppresses Bax/Bak Conformational Change and Mitochondrial

Apoptosis. Mol. Cell Biol. 25: 9369-9382, 2005.

120. House, S.B., Rusnak, M., Liu, X-H., Youle, R.J. and H. Gainer. Bcl-xL and caspase

inhibition increase the survival of rat oxytocin and vasopressin magnocellular neurons in

organotypic culture. Exp. Neurol. 200:267-71, 2006.

121. Barsoum MJ, Yuan H, Gerencser AA, Liot G, Kushnareva Y, Graber S, Kovacs I, Lee

WD, Waggoner J, Cui J, White AD, BossyB, Martinou JC, Youle RJ, Lipton SA,

Ellisman MH, Perkins GA, Bossy-Wetzel E. Nitric oxide-induced mitochondrial fission

is regulated by dynamin-related GTPases in neurons. EMBO J. 25: 3900-3911, 2006.

122. Karbowski, M., Norris, K., Jeong, S.Y., Cleland, M., and Youle R.J. Role of Bax and

Bak in mitochondrial morphogenesis. Nature 443: 658-662, 2006. Commentaries in Cell

and Nature.

123. Antignani, A. and Youle R.J. The cytokine, GM-CSF, can deliver Bcl-XL as an

extracellular fusion protein to protect cells from apoptosis and retain differentiation

induction. J. Biol. Chem. 282: 11246-11254, 2007.

124. Goyal, G., Fell, B., Goyal, G., Youle R.J. and V. Sriram. Mitochondrial remodeling

during programmed cell death in Drosophila. Dev. Cell 12: 807-816, 2007. Highlighted

in Nature.

125. Karbowski, M., Neutzner, A. and Youle R.J. The mitochondrial E3 ubiquitin ligase,

MARCH5, is required for Drp1 dependent mitochondrial division. J. Cell Biol. 178: 71-

84 2007. Commentary in J. Cell Biol.

126. Lee, S., Jeong, S-Y, Lim, W-C., Kim, S., Park, Y-Y. Sun, X., Youle, R.J., and Cho, H.

Mitochondrial fission and fusion mediators, hFis1 and OPA1, modulate cellular

senescence. J. Biol. Chem. 282: 22977-83, 2007.

127. Zanna C, Ghelli A, Porcelli AM, Karbowski M, Youle RJ, Schimpf S, Wissinger B, Pinti

M, Cossarizza A, Vidoni S, Valentino ML, Rugolo M, Carelli V. OPA1 mutations

associated with dominant optic atrophy impair oxidative phosphorylation and

mitochondrial fusion. Brain 131:352-67, 2008.

128. Antignani, A. and Youle R.J. Endosome Fusion Induced by Diphtheria Toxin

Translocation Domain. Proc. Natl. Acad. Sci. USA 105: 8020-5, 2008.

129. Norris, K.R. and Youle, R.J. Cytomegalovirus proteins vMIA and m38.5 link

mitochondrial morphogenesis to Bcl-2 family proteins.

J Virol. 82:6232-6243, 2008.

130. Narendra D, Tanaka A, Suen DF, Youle RJ. Parkin is recruited selectively to impaired

mitochondria and promotes their autophagy.

J Cell Biol. 183:795-803, 2008.

Commentaries in Nature Cell Biol., Science Signaling

131. Tondera, D., Grandemange, S., Jourdain, A., Karbowski, M., Mattenberger, Y.,Herzig, S., Clerc,

P., Da Cruz, S., Raschke, I., Merkwirth, C., Ehses, S., Krause, F., Chan, D.C., Alexander, C.,

Bauer, C., Youle R.J., Langer, T. and Martinou, JC. SLP-2 is required for stress-induced

mitochondrial hyperfusion. EMBO J. 28:1589-1600, 2009.

132. Narendra D, Tanaka A, Suen DF, Youle RJ. Parkin-induced mitophagy in the pathogenesis of

Parkinson's disease. Autophagy, 5: 706-708 2009.

133. Galluzzi, L., S. Aaronson, J. Abrams, E. Alnemri, D. Andrews, A. Ashkenazi, E. Baehrecke, N. Bazan, M. Blagosklonny, K. Blomgren, C. Borner, D. Bredesen, C. Brenner, M. Castedo, J. Cidlowski, A. Ciechanover, G. Cohen, Vincenzo De Laurenzi, R. De Maria, M. Deshmukh, B. Dynlacht, W. El-Deiry, R. Flavell, S. Fulda, C. Garrido, P. Golstein, M. Gougeon, D. Green, H. Gronemeyer, G. Hajnóczky, J. Hardwick, M. Hengartner, H. Ichijo, M. Jäättelä, O. Kepp, A. Kimchi, D. Klionsky, R. Knight, S. Kornbluth, S. Kumar, B. Levine, S. Lipton, E. Lugli, F. Madeo, W. Malorni, J. Marine, S. Martin, J. Medema, P. Mehlen, G. Melino, U. Moll, E. Morselli, S. Nagata, D. Nicholson, P. Nicotera, G. Nuñez, M. Oren, J. Penninger, S. Pervaiz, M. Peter, M. Piacentini, J. Prehn, H. Puthalakath, G. Rabinovich, R. Rizzuto, C. Rodrigues, D. Rubinsztein, T. Rudel, L. Scorrano, H. Simon, H. Steller, J. Tschopp, Y. Tsujimoto, P. Vandenabeele, I. Vitale, K. Vousden, R. Youle, J. Yuan, B. Zhivotovsky and G. Kroemer,. Guidelines for

the use and interpretation of assays for monitoring cell death in higher eukaryotes. Cell

Death Differ. 16:1093-107, 2009

134. Rostovtseva TK, Boukari H, Antignani A, Shiu B, Banerjee S, Neutzner A, Youle RJ.

Bax activates endophilin B1 oligomerization and lipid membrane vesiculation.J. Biol.

Chem. 284: 34390-34399, 2009.

135. Narendra D, Jin

S, Tanaka

A, Suen

DF, Gautier

C, Shen

J, Cookson

M, Youle

RJ.

PINK1 is selectively stabilized on impaired mitochondria to trigger Parkin recruitment.

PLoS Biol., 8, e1000298 (2010).

Commentaries in Nature, PLoS Biol., Nature Reviews Neurology

136. Benard G, Neutzner A, Peng G,

Wang C., Livak F, Youle RJ, Karbowski M.

IBRDC2, an IBR-type E3 ubiquitin ligase, is a regulatory factor for Bax and apoptosis

activation. EMBO J. 29:1458-71, 2010.

137. Park YY, Lee S, Karbowski M, Neutzner A, Youle RJ, Cho H. Loss of MARCH5

mitochondrial E3 ubiquitin ligase induces cellular senescence through dynamin-related

protein 1 and mitofusin 1. J Cell Sci. 123:619-26, 2010.

138. Du X, Youle RJ, Fitzgerald DJ, Pastan I. Pseudomonas Exotoxin A-mediated Apoptosis

is Bak- dependent and Preceded by the Degradation of Mcl-1. Mol Cell Biol. 30: 3444-

3452, 2010.

139. Suen D, Narendra D, Tanaka A, Manfredi G, Youle RJ. Parkin overexpression selects

against a deleterious mtDNA mutation in heteroplasmic cybrid cells. Proc. Natl. Acad.

Sci. USA 107: 11835-11840, 2010. Highlighted in PNAS.

140. Jin SM, Lazarou M, Wang C, Kane LA, Narendra DP, Youle RJ. Mitochondrial

membrane potential regulates PINK1 import and proteolytic destabilization by PARL.

J. Cell Biol. 191: 933-942, 2010.

141. Narendra DP, Kane LA, Hauser DN, Fearnley IM, Youle RJ. p62/SQSTM1 is required

for Parkin-induced mitochondrial clustering but not mitophagy; VDAC1 is dispensable

for both. Autophagy 6: 1090-1106, 2010. Commentary in F1000.

142. Tanaka A, Cleland MM, Shan Xu S, Narendra DP, Suen DF, Mariusz Karbowski M,

Youle RJ. Proteasome and p97 mediate mitophagy and degradation of mitofusins

induced by Parkin. J. Cell Biol. 191: 933-942, 2010. Commentary in JCB, F1000.

143. Otera H, Wang C, Cleland ME, Setoguchi K, Yokota S, Youle RJ, Mihara K.

Mff is an essential factor for mitochondrial recruitment of Drp1 during mitochondrial

fission in mammalian cells. J. Cell Biol. 191: 1141-1158, 2010.

144. Hoppins S, Edlich F, Cleland MM, Banerjee S, McCaffery JM, Youle RJ, Nunnari

J. The

soluble form of Bax regulates mitochondrial fusion via MFN2 homotypic complexes.

Mol. Cell, 144: 50-160, 2011. Commentary in Dev. Cell, F1000

145. Neutzner, A, Neutzner, M, Benischke, A, Ryu, S-W, Frank, S, Youle, RJ, Karbowski, M.

A systematic search for ER membrane-associated RING finger proteins identifies

Nixin/ZNRF4 as a regulator of calnexin stability and ER homeostasis. J. Biol. Chem.

286: 8633-8643, 2011.

146. Edlich F, Banerjee S, Suzuki M, Arnoult D, Cleland M, Wang C, Neutzner A, Tjandra N,

Youle RJ. Bcl-xL retrotranslocates Bax from mitochondria into the cytosol.

Cell, 145: 104-116, 2011. Commentary in Cell, F1000.

147. Carelli V, Schimpf S, Fuhrmann N, Valentino ML, Zanna C, Iommarini L, Papke M,

Schaich S, Tippmann S, Baumann B, Barboni P, Longanesi L, Rugolo M, Ghelli A, Alavi

M, Youle R, Bucchi L, Carroccia R, Giannoccaro MP, Tonon C, Lodi R, Cenacchi G,

Montagna P, Liguori R, Wissinger B. Hum Mol Genet. 20: 1893-1905, 2011

148. Kim KY, Stevens MV, Rusk SE, Huang RJ, Cohen A, Noguchi A, Springer D, Bocharov

AV, Eggerman TL, Suen DF, Youle RJ, Amar M, Remaley AT, Sack MN. Parkin is a

lipid-responsive regulator of fat intake in mice and mutant human cells. J. Clin. Invest.

121: 3701-3712, 2011.

149. Joo JH, Dorsey FC, Joshi A, Hennessy-Walters KM, Rose KL, McCastlain K, Zhang J,

Iyengar R, Jung CH, Suen DF, Steeves MA, Yang CY, Prater SM, Kim DH, Thompson

CB, Youle RJ, Ney PA, Cleveland JL, Kundu M. Hsp90-Cdc37 Chaperone Complex

Regulates Ulk1- and Atg13- mediated mitophagy. Mol. Cell 43: 572-585, 2011.

150. Wang C, and Youle RJ. Predominant requirement of Bax for apoptosis in HCT116 cells

is determined by Mcl-1's inhibitory effect on Bak. Oncogene doi: 10.1038/onc.2011.497,

2011.

151. Perciavalle RM, Stewart

DP, Koss

B, Lynch

J, Milasta

S, Bathina

M, Cleland

MM,

Pelletier S, Schuetz

JD, Youle

RJ, Green

DR, and Opferman

JT. Anti-Apoptotic MCL-1

Localizes to the Mitochondrial Matrix and Couples Mitochondrial Fusion to Respiration.

Nature Cell Biology in press, 2012

152. Sims J, Scavone F, Cooper E, Kane L, Youle RJ, Boeke J, Cohen R. Polyubiquitin-sensor

proteins reveal localization and linkage-type dependence of cellular ubiquitin signaling.

Nature Methods in press 2012.

153. Lazarou M, Jin SM, Kane L, Youle RJ. Role of PINK1 binding to the TOM

complex and alternate intracellular membranes in recruitment and activation of the E3

ligase Parkin. Dev. Cell in press, 2012.

Chapters and Invited Reviews and Editorials

1. Neville DM, Jr., Murray GJ and Youle RJ: Directed entry of hybrid proteins into cells via alternate receptors. Biochem Soc Trans 8:692-693, 1980.

2. Neville DM, Jr. and Youle RJ: Monoclonal antibody-ricin A chain hybrids: Kinetic analysis of cell killing for tumor therapy. Immunological Reviews 62:135-151, 1981.

3. Neville DM, Jr., Heagy W, Murray GJ and Youle RJ: Receptor mediated transport of hybrid proteins:

Therapeutic possibilities and attempts to delineate entry mechanism. Symposium on Receptor Mediated

Binding and Internalization of Toxins and Hormones . Academic Press, 329-338, 1981.

4. Youle RJ and Neville DM, Jr.: Hybrid proteins used to study the mechanism of toxin entrance into cells. In: Weinstein L and Fields DN (eds..) Seminars in Infectious Diseases, IV: Bacterial Vaccines, Brain C.

Decker, pp. 86-88, 1982.

5. Vallera DA, Kersey JH, Quinones RR, Zanjani ED, Soderling CCB, Azemove SM, LeBien TW, Beverley

PCL, Ash RC, Neville DM,, Jr. and Youle RJ: Antibody-ricin conjugates: purgative reagents for murine

and human allogeneic bone marrow transplantation. In: Recent Advances in Bone Marrow

Transplantation New Series, Vol. 7. Gale RP (ed), AR Liss. Inc., New York, 1983

6. Neville DM, Jr, Youle RJ, Kersey JH and Vallera DA: Monoclonal antibody-ricin conjugates for the

treatment of graft versus host disease. Present and future prospects. In: The Armand Hammer Cancer

Symposium, Monoclonal Antibodies and Cancer. Langman R and Dulbecco R (eds.), Academic Press, pp.

107-116, 1983.

7. Stong RC, Youle RJ, Kersey JH, Zanjani Ed and Vallera: Use of cocktail of monoclonal antibody-intact

ricin conjugates to purge human bone marrow. Proc. First Internat Symposium on Autologous Bone

Marrow Transplantation Kicke K, Spitzer G, Zander A and Gorin N (eds), Gulf Coast Printing 1983.

8. Vallera DA, Youle RJ, Filipovich AH, Neville DM, Zanjani ED, Soderling CCB, Azemove SM and Kersey

JH: Immunotoxins and allogeneic bone marrow transplantation for treatment of human leukemia and

lymphoma. In: New Raven Press , NY, pp. 397-405, 1984.

9. Youle RJ and Neville DM, Jr: Long-term kinetics of antibody-toxin conjugates show resistant cells. In:

Receptor-Medicated Targeting of Drugs Gregoriadis G, Poste G, Senior J and Trouet A (eds.), Plenum Publishing Co., pp. 139-145, 1985.

10. Filipovich AH, Vallera DA, Youle RJ, Neville DM, Jr., Kersey JH: Ex vivo T cell depletion with

immunotoxins in allogeneic bone marrow transplantation: The pilot clinical study for prevention of graft-versus-host disease. Transplantation Proceedings 17:442-444, 1985.

11. Kersey JH, Vallera D, Ramsay N, Filipovich A, Youle R, Neville D, Stong R and LeBien T: Monoclonal antibodies and immunotoxins in bone marrow transplantation: Purging marrow of

ALL or GVHD cells with preservation of stem cells. Haematology and Blood Transfusion

29:34-38, 1985.

12. Youle RJ and Colombatti M: Immunotoxins: Monoclonal antibodies antibodies linked to toxin proteins for

bone marrow transplantation and cancer therapy. In: Monoclonal Antibodies in Cancer: Advances in

Diagnosis and Treatment Roth JA (ed), Mount Kisco, N.Y., Futura Publishing Co., pp. 173-213, 1986.

13. Colombatti M, Greenfield L and Youle RJ: Antibody-toxin conjugates help clarify surface antigen behavior

and toxin entry mechanisms. In: Membrane Mediated Cytotoxicity , UCLA Symposium on Molecular and Cellular Biology, New Series. Vol. 45, Bonavida B and Collier RJ (eds.). Alan Liss Inc., NY, pp. 155-165,

1987.

14. Youle RJ and Neville DM, Jr.: Role of endocytosis and receptor recycling in ligand-toxin and antibody-toxin conjugate activity. In: Immunoconjugates: Antibody Conjugates in Radioimaging and Therapy of

Cancer Vogel CW (ed.), Oxford University Press, pp. 153-169, 1987.

15. Youle RJ: Editorial: Fatal attraction in vitro and in vivo. J Natl Cancer Inst 80:388-389, 1988.

16. Youle RJ, Johnson VG and Greenfield L: Genetic engineering of immunotoxins. In: Immunotoxins . Frankel A (ed.) Kluwer Academic Publishers, pp. 113-122, 1988.

17. Youle RJ: Toxin selection and modification - Overview. In: Immunotoxins. Frankel A (ed.) Kluwer

Academic Publishers. pp. 93-96, 1988.

18. Weber RJ and Youle RJ: Immunological approaches to the identification of cell surface specific antigens in

brain. In: Neurobiological Research Marangos PJ, Campbell I and Cohen (eds.) Academic Press, Inc.

(pp. 51-67), 1988.

19. Bridges S, Longo DL, and Youle RJ: The preparation and use of anti-idiotypic antibodies armed with

holotoxins or hemitoxins in the treatment of B-cell neoplasms. Methods in Enzymology 178:356-368, 1989.

20. Rybak S. and Youle RJ: Immunotoxins. Immunology and Clinics of North America 11:359-380, 1991.

21. Youle RJ: Mutations in Diphtheria Toxin to Improve Immunotoxin Selectivity and Understand Toxin Entry

into Cells. Seminars in Cell Biology 2:39-45, 1991.

22. Nicholls P. and Youle RJ. Structure Function Relationships in Diphtheria Toxin. Targeted Diagn Ther

7:439-446, 1991.

23. Nicholls P. and Youle RJ: Structure Function Relationships in Pseudomomas Exotoxin A. Targeted Diagn

Ther 7:339-363, 1991.

24. Youle RJ, Newton DL, Wu Y-N, Gadina M, and Rybak SM: Cytotoxic Ribonucleases and Chimeras in

Cancer Therapy. Critical Reviews in Therapeutic Drug Carrier Syst 10:1-28, 1993.

25. Johnson VG and Youle RJ: Intracellular routing and membrane translocation of diphtheria toxin and ricin. In: Intracellular Trafficking of Proteins, Steer CJ and Hanover JA (eds.). Cambridge University Press. pp

183-225,1991.

26. Rybak SM, Hoogenboom, HR, Newton, DL, Raus, JCM, and RJ Youle, Rational Immunotherapy with

Ribonuclease Chimeras: An Approach Toward Humanizing Immunotoxins. In:Applications of Monoclonal

Antibodies in Cancer Therapy, Cell Biophys 21:121-138, 1992.

27. Rybak SM, Newton DL, Mikulski SM, Viera A.. and Youle RJ: Cytotoxic Onconase and Ribonuclease

Chimeras: Comparison and In Vitro Characterization. Drug Targeting and Delivery 1:3-10, 1993.

28. Gadina M, Newton DL, Rybak SM, Wu Y-N and Youle RJ: Humanized Immunotoxins. Therap Immun

1:59-64, 1994.

29. Wood KA and Youle RJ: Apoptosis and Free Radicals. In: The Neurobiology of NO

. and

.OH. NY Acad of

Sci 738:400-407,1994

30. Illercil O, Laske DW, Walbridge S, Muraszko KM, Oldfield EH and Youle RJ: Neurotoxicity of Immunotoxins. Molecular and Chemical Neuropathology 21:379-386, 1994

31. Youle RJ: Immunotoxins for Central Nervous System Malignancy. Seminars in Cancer Biology 7:65-70, 1996.

32. Youle RJ and D'Alessio G: Antitumor Ribonucleases. In: Ribonucleases: Structure and Function. Riordan J and D'Alessio G, Academic Press, 491-514, 1996.

33. Oldfield EH and Youle RJ: Immunotoxins for Brain Tumor Therapy. In: Clinical Applications of

Immunotoxins. in press, 1997

34. Hsu, Y-T and Youle RJ: Bax, a death-effector molecule: its role in development and

oncogenesis. Chemokines and Cancer Therapy. 1998.

35. Huang AHC and Youle RJ: The discovery of 2S albumins as abundant storage proteins in seeds.

Discoveries in Plant Biology III, 255-266, 1999.

36. Youle RJ: Immunotoxins for Brain Tumor Therapy. Brain Barrier Systems, Alfred Benzon Symposium

45, 225-230, 1999

37. Suzuki M and Youle RJ: De nouvelles “balles magiques” contre le cancer. Biofuture 198: 34-37, 2000.

38. Smaili SS, Hsu YT, Youle RJ, Russell JT. Mitochondria in Ca2+ signaling and apoptosis.

J Bioenerg Biomembr. 32(1):35-46, 2000.

39. Youle, R.J. Molecular Neurosurgery with Targeted Toxins. Eds. R.G. Wiley and D.A. Lappi, Humana

Press, 2003 in press.

40. Youle, R.J. Book Review: “Chimeric Toxins: Mechanisms of Action and Therapeutic Applications”, The

International Journal of Toxicology, 2003, in press.

41. Robert, E., Frank, S., and Youle R.J. Scission, Spores and Apoptosis, a proposal for the evolutionary

origin of mitochondria in cell death induction. BBRC, 304: 481-486, 2003.

42. Sharpe, J., Arnoult, D., and Youle R.J., Regulation of Mitochondrial Permeability by Members of the Bcl-2

Family. Biochem. Biophys Acta, 2003, in preparation.

43. Karbowski, M. and Youle R.J. The Dynamics of Mitochondrial Morphology in Healthy Cells and During

Apoptosis. Cell Death and Differentiation 10: 870-880, 2003.

44. Smaili SS, Hsu YT, Carvalho AC, Rosenstock TR, Sharpe JC, Youle RJ. Mitochondria, calcium and pro-apoptotic proteins as mediators in cell death signaling. Braz J Med Biol Res. 36:183-190, 2003.

45. Arnoult D., Karbowski, M. and Youle RJ. Caspase inhibition prevents the mitochondrial release of apoptosis-inducing factor. Cell Death and Differentiation 10:845-849, 2003.

46. Sharpe, J., Arnoult, D., and Youle R.J., Control of Mitochondrial Permeability by Bcl-2 Family Members. Biochem. Biophys. Acta., 1644: 107-113, 2004.

47. Youle R.J. Role of Mitochondria in Apoptosis: Worms-to-Beetles in Worms.

Dev. Cell 8:298-299, 2005.

48. Youle R.J. and Karbowski, M. Apoptosis Involves Mitochondrial Fission.

Nature Rev. Mol. Cell Biol. 6: 657-663, 2005.

49. Antignani A and Youle R.J. How do Bax and Bak lead to permeabilization of the outer mitochondrial

membrane? Curr. Opin. Cell Biol. 18: 685-689, 2006.

50. Martinou JC, Youle RJ. Which came first, the cytochrome c release or the mitochondrial

fission? Cell Death Differ. 13:1291-1295, 2006.

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