Richard J Youle

Summary

Affiliation: National Institutes of Health
Country: USA

Publications

  1. pmc Mitochondrial release of AIF and EndoG requires caspase activation downstream of Bax/Bak-mediated permeabilization
    Damien Arnoult
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    EMBO J 22:4385-99. 2003
  2. ncbi request reprint Cell biology. Cellular demolition and the rules of engagement
    Richard J Youle
    Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Science 315:776-7. 2007
  3. pmc Predominant requirement of Bax for apoptosis in HCT116 cells is determined by Mcl-1's inhibitory effect on Bak
    C Wang
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Oncogene 31:3177-89. 2012
  4. doi request reprint Mitochondrial fission, fusion, and stress
    Richard J Youle
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Science 337:1062-5. 2012
  5. doi request reprint Mechanisms of mitophagy
    Richard J Youle
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 35 Convent Drive, 2C 917, Bethesda, Maryland 20892, USA
    Nat Rev Mol Cell Biol 12:9-14. 2011
  6. ncbi request reprint The BCL-2 protein family: opposing activities that mediate cell death
    Richard J Youle
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, The National Institutes of Health, Bethesda, Maryland 20892, USA
    Nat Rev Mol Cell Biol 9:47-59. 2008
  7. ncbi request reprint Mitochondrial fission in apoptosis
    Richard J Youle
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
    Nat Rev Mol Cell Biol 6:657-63. 2005
  8. pmc Proteasome and p97 mediate mitophagy and degradation of mitofusins induced by Parkin
    Atsushi Tanaka
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    J Cell Biol 191:1367-80. 2010
  9. ncbi request reprint Novel structure of the N terminus in yeast Fis1 correlates with a specialized function in mitochondrial fission
    Motoshi Suzuki
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 280:21444-52. 2005
  10. pmc Roles of the mammalian mitochondrial fission and fusion mediators Fis1, Drp1, and Opa1 in apoptosis
    Yang ja Lee
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Mol Biol Cell 15:5001-11. 2004

Collaborators

Detail Information

Publications69

  1. pmc Mitochondrial release of AIF and EndoG requires caspase activation downstream of Bax/Bak-mediated permeabilization
    Damien Arnoult
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    EMBO J 22:4385-99. 2003
    ..Thus EndoG and AIF seem to define a 'caspase-dependent' mitochondria-initiated apoptotic DNA degradation pathway that is conserved between mammals and nematodes...
  2. ncbi request reprint Cell biology. Cellular demolition and the rules of engagement
    Richard J Youle
    Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Science 315:776-7. 2007
  3. pmc Predominant requirement of Bax for apoptosis in HCT116 cells is determined by Mcl-1's inhibitory effect on Bak
    C Wang
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Oncogene 31:3177-89. 2012
    ..Our data suggest that Bax and Bak are functionally redundant, but they are counteracted by distinct anti-apoptotic Bcl-2 family proteins in different species...
  4. doi request reprint Mitochondrial fission, fusion, and stress
    Richard J Youle
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Science 337:1062-5. 2012
    ..Disruptions in these processes affect normal development, and they have been implicated in neurodegenerative diseases, such as Parkinson's...
  5. doi request reprint Mechanisms of mitophagy
    Richard J Youle
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 35 Convent Drive, 2C 917, Bethesda, Maryland 20892, USA
    Nat Rev Mol Cell Biol 12:9-14. 2011
    ..Moreover, mitophagy is regulated in many metazoan cell types by parkin and PTEN-induced putative kinase protein 1 (PINK1), and mutations in the genes encoding these proteins have been linked to forms of Parkinson's disease...
  6. ncbi request reprint The BCL-2 protein family: opposing activities that mediate cell death
    Richard J Youle
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, The National Institutes of Health, Bethesda, Maryland 20892, USA
    Nat Rev Mol Cell Biol 9:47-59. 2008
    ..Although these insights into interactions among BCL-2 family proteins reveal how these proteins are regulated, a unifying hypothesis for the mechanisms they use to activate caspases remains elusive...
  7. ncbi request reprint Mitochondrial fission in apoptosis
    Richard J Youle
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
    Nat Rev Mol Cell Biol 6:657-63. 2005
    ..One of the steps in apoptosis is the fragmentation of mitochondria, and recent evidence indicates that the mitochondrial fission machinery actively participates in the process of programmed cell death...
  8. pmc Proteasome and p97 mediate mitophagy and degradation of mitofusins induced by Parkin
    Atsushi Tanaka
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    J Cell Biol 191:1367-80. 2010
    ..Inhibition of Drp1-mediated mitochondrial fission, the proteasome, or p97 prevents Parkin-induced mitophagy...
  9. ncbi request reprint Novel structure of the N terminus in yeast Fis1 correlates with a specialized function in mitochondrial fission
    Motoshi Suzuki
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 280:21444-52. 2005
    ..Although the TPR-like helix bundle of Fis1 mediates the interaction with Dnm1 and Mdv1, the N terminus of Fis1 is a prerequisite to recruit Mdv1 to facilitate mitochondrial fission...
  10. pmc Roles of the mammalian mitochondrial fission and fusion mediators Fis1, Drp1, and Opa1 in apoptosis
    Yang ja Lee
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Mol Biol Cell 15:5001-11. 2004
    ..However, we provide further evidence that multiple components of the mitochondrial morphogenesis machinery can positively and negatively regulate apoptosis...
  11. pmc Parkin is recruited selectively to impaired mitochondria and promotes their autophagy
    Derek Narendra
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    J Cell Biol 183:795-803. 2008
    ..These results show that Parkin promotes autophagy of damaged mitochondria and implicate a failure to eliminate dysfunctional mitochondria in the pathogenesis of Parkinson's disease...
  12. pmc Bcl-x(L) retrotranslocates Bax from the mitochondria into the cytosol
    Frank Edlich
    Surgical Neurology Branch, NINDS, National Institutes of Health, Bethesda, MD 20892, USA
    Cell 145:104-16. 2011
    ..We propose that Bcl-x(L) inhibits and maintains Bax in the cytosol by constant retrotranslocation of mitochondrial Bax...
  13. pmc The mitochondrial E3 ubiquitin ligase MARCH5 is required for Drp1 dependent mitochondrial division
    Mariusz Karbowski
    Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20852, USA
    J Cell Biol 178:71-84. 2007
    ..Collectively, our data suggest a model in which mitochondrial division is regulated by a MARCH5 ubiquitin-dependent switch...
  14. pmc Cytomegalovirus cell death suppressor vMIA blocks Bax- but not Bak-mediated apoptosis by binding and sequestering Bax at mitochondria
    Damien Arnoult
    National Institute of Neurological Disorders and Stroke Biochemistry Section, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 101:7988-93. 2004
    ....
  15. pmc Endophilin B1 is required for the maintenance of mitochondrial morphology
    Mariusz Karbowski
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bldg 35, Rm 917, MSC 3407, 35 Lincoln Drive, Bethesda, MD 20892 1414, USA
    J Cell Biol 166:1027-39. 2004
    ....
  16. pmc PINK1 drives Parkin self-association and HECT-like E3 activity upstream of mitochondrial binding
    Michael Lazarou
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    J Cell Biol 200:163-72. 2013
    ..Self-association occurred independent of ubiquitination activity through the RING-between-RING domain, providing mechanistic insight into how PINK1 activates Parkin...
  17. doi request reprint High-content genome-wide RNAi screens identify regulators of parkin upstream of mitophagy
    Samuel A Hasson
    1 Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA 2 Division of Preclinical Innovation, National Center for Advancing Translational Sciences, National Institutes of Health, Rockville, Maryland 20850, USA 3
    Nature 504:291-5. 2013
    ..Overall, our screens provide a rich resource to understand mitochondrial quality control. ..
  18. pmc Quantitation of mitochondrial dynamics by photolabeling of individual organelles shows that mitochondrial fusion is blocked during the Bax activation phase of apoptosis
    Mariusz Karbowski
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    J Cell Biol 164:493-9. 2004
    ..The block in mitochondrial fusion occurs within the same time range as Bax coalescence on the mitochondria and outer mitochondrial membrane permeabilization, and it may be a consequence of Bax/Bak activation during apoptosis...
  19. pmc Structural mechanism of Bax inhibition by cytomegalovirus protein vMIA
    Junhe Ma
    Laboratory of Molecular Biophysics, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 109:20901-6. 2012
    ..The structure suggests that by stabilizing key elements in Bax needed to unravel for its MOM insertion and oligomerization, vMIA prevents these important steps in apoptosis...
  20. pmc Parkin overexpression selects against a deleterious mtDNA mutation in heteroplasmic cybrid cells
    Der Fen Suen
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 107:11835-40. 2010
    ..These data support the model that Parkin functions in a mitochondrial quality control pathway. Additionally, they suggest that transiently increasing levels of Parkin expression might ameliorate certain mitochondrial diseases...
  21. pmc Role of PINK1 binding to the TOM complex and alternate intracellular membranes in recruitment and activation of the E3 ligase Parkin
    Michael Lazarou
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Dev Cell 22:320-33. 2012
    ..We propose that the association of PINK1 with the TOM complex allows rapid reimport of PINK1 to rescue repolarized mitochondria from mitophagy, and discount mitochondrial-specific factors for Parkin translocation and activation...
  22. pmc Bax activates endophilin B1 oligomerization and lipid membrane vesiculation
    Tatiana K Rostovtseva
    Laboratory of Physical and Structural Biology, Eunice Kennedy Shriver NICHD, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 284:34390-9. 2009
    ..This activity of purified Bax protein to induce cell-free assembly of Endo B1 may reflect its activity in cells that regulates apoptosis and/or mitochondrial fusion...
  23. pmc Role of membrane association and Atg14-dependent phosphorylation in beclin-1-mediated autophagy
    Adam I Fogel
    Biochemistry Section, National Institute for Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA
    Mol Cell Biol 33:3675-88. 2013
    ..We map these novel phosphorylation sites to serines 90 and 93 and demonstrate that phosphorylation at these sites is necessary for maximal autophagy. These results help clarify the mechanism of beclin-1 and Atg14 during autophagy. ..
  24. pmc Bcl-x(L) sequesters its C-terminal membrane anchor in soluble, cytosolic homodimers
    Seon Yong Jeong
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 1414, USA
    EMBO J 23:2146-55. 2004
    ..The C-terminal tail of Bcl-x(L) is also required to mediate Bcl-x(L)/Bax heterodimer formation. Both mitochondrial import and antiapoptotic activity of different Bcl-x(L) mutants correlate with their ability to form homodimers...
  25. ncbi request reprint Control of mitochondrial permeability by Bcl-2 family members
    Juanita C Sharpe
    Biochemistry Section, Surgical Neurology Branch, NINDS, NIH, Bethesda, MD 20892, USA
    Biochim Biophys Acta 1644:107-13. 2004
    ..The molecular mechanisms of the different models for the permeabilization of membranes by the Bcl-2 family members and the regulation of Bcl-2 family member subcellular localizations are discussed...
  26. ncbi request reprint The solution structure of human mitochondria fission protein Fis1 reveals a novel TPR-like helix bundle
    Motoshi Suzuki
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    J Mol Biol 334:445-58. 2003
    ....
  27. pmc p62/SQSTM1 is required for Parkin-induced mitochondrial clustering but not mitophagy; VDAC1 is dispensable for both
    Derek Narendra
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke National Institutes of Health, Bethesda, MD, USA
    Autophagy 6:1090-106. 2010
    ..They also suggest that proteins other than p62 are likely required for mitophagy downstream of Parkin substrates other than VDAC1...
  28. ncbi request reprint Outer mitochondrial membrane protein degradation by the proteasome
    Albert Neutzner
    Biochemistry Section, SNB, NINDS, NIH, Bethesda, MD 20892, USA
    Novartis Found Symp 287:4-14; discussion 14-20. 2007
    ..Some of these mitochondrial RING domain proteins also regulate mitochondrial morphology, indicating a critical role of ubiquitin signalling in the maintenance of mitochondrial homeostasis...
  29. pmc Mitochondrial dynamics and apoptosis
    Der Fen Suen
    Biochemistry Section, Surgical Neurology Branch, NINDS, National Institutes of Health, Bethesda, MD 20892, USA
    Genes Dev 22:1577-90. 2008
    ..This review will cover the recent advances and presents competing models on how the mitochondrial fission and fusion machinery may intersect apoptosis pathways...
  30. ncbi request reprint A chimeric protein induces tumor cell apoptosis by delivering the human Bcl-2 family BH3-only protein Bad
    Antonella Antignani
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892 3704, USA
    Biochemistry 44:4074-82. 2005
    ..The completely human sequence and the elevated selectivity for cancer cells could prevent immunogenicity and the nonspecific toxicity of targeted toxins in future clinical application of this fusion protein...
  31. ncbi request reprint Parkin-induced mitophagy in the pathogenesis of Parkinson disease
    Derek Narendra
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Autophagy 5:706-8. 2009
    ..These findings suggest that Parkin promotes mitophagy of dysfunctional mitochondria following loss of mitochondrial membrane potential and implicates the targeted elimination of mitochondria in the pathogenesis of Parkinson disease...
  32. pmc A systematic search for endoplasmic reticulum (ER) membrane-associated RING finger proteins identifies Nixin/ZNRF4 as a regulator of calnexin stability and ER homeostasis
    Albert Neutzner
    Biochemistry Section, Surgical Neurological Branch, NINDS, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 286:8633-43. 2011
    ..Importantly, Nixin physically interacts with calnexin in a glycosylation-independent manner, induces calnexin ubiquitination, and p97-dependent degradation, indicating an ER-associated degradation-like mechanism of calnexin turnover...
  33. pmc PINK1 is selectively stabilized on impaired mitochondria to activate Parkin
    Derek P Narendra
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA
    PLoS Biol 8:e1000298. 2010
    ..In addition, they support a novel model for the negative selection of damaged mitochondria, in which PINK1 signals mitochondrial dysfunction to Parkin, and Parkin promotes their elimination...
  34. pmc Endosome fusion induced by diphtheria toxin translocation domain
    Antonella Antignani
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 35 Convent Drive MSC 3704, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 105:8020-5. 2008
    ..These changes to endosomes may reflect activities of the T domain that mediate toxin entry to the cytosol. The nontoxic mutant DT, CRM197, yields a new tool to manipulate endosome dynamics in living cells...
  35. pmc Mitochondrial membrane potential regulates PINK1 import and proteolytic destabilization by PARL
    Seok Min Jin
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    J Cell Biol 191:933-42. 2010
    ..Thus, differential localization to the inner and outer mitochondrial membranes appears to regulate PINK1 stability and function...
  36. ncbi request reprint Instability of the mitofusin Fzo1 regulates mitochondrial morphology during the mating response of the yeast Saccharomyces cerevisiae
    Albert Neutzner
    Biochemistry Section, Surgical Neurological Branch, NINDS, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 280:18598-603. 2005
    ..Proteasomal degradation of Fzo1 in response to the mating pheromone is proposed to mediate the remodeling of the mitochondrial network during the process of mating...
  37. ncbi request reprint Bid, but not Bax, regulates VDAC channels
    Tatiana K Rostovtseva
    Laboratory of Physical and Structural Biology, NICHD, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 279:13575-83. 2004
    ..We speculate that by decreasing the probability of VDAC opening, Bid reduces metabolite exchange between mitochondria and the cytosol, leading to mitochondrial dysfunction...
  38. pmc Spatial and temporal association of Bax with mitochondrial fission sites, Drp1, and Mfn2 during apoptosis
    Mariusz Karbowski
    Biochemistry Section, SNB, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    J Cell Biol 159:931-8. 2002
    ..Surprisingly, Drp1 and Mfn2, but not other proteins implicated in the regulation of mitochondrial morphology, colocalize with Bax in these foci. We suggest that Bax participates in apoptotic fragmentation of mitochondria...
  39. pmc Ubiquitin ligase RNF167 regulates AMPA receptor-mediated synaptic transmission
    Marc P Lussier
    Receptor Biology Section and Biochemistry Section Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 109:19426-31. 2012
    ..Therefore, our study identifies RNF167 as a selective regulator of AMPAR-mediated neurotransmission and expands our understanding of how ubiquitination dynamically regulates excitatory synapses...
  40. pmc Pseudomonas exotoxin A-mediated apoptosis is Bak dependent and preceded by the degradation of Mcl-1
    Xing Du
    Laboratory of Molecular Biology, National Cancer Institute, 37 Convent Drive, Bethesda, MD 20892 4264, USA
    Mol Cell Biol 30:3444-52. 2010
    ..Overexpression of Mcl-1 and Bcl-x(L) inhibited PE-induced MEF death. Our data suggest that Bak is the preferential mediator of PE-mediated apoptosis and that the rapid degradation of Mcl-1 unleashes Bak to activate apoptosis...
  41. doi request reprint The role of mitochondria in apoptosis*
    Chunxin Wang
    Biochemistry Section, Surgical Neurology Branch, NINDS, National Institutes of Health, Bethesda, Maryland 20892, USA
    Annu Rev Genet 43:95-118. 2009
    ..We also compare and contrast apoptosis pathways in Caenorhabditis elegans, Drosophila melanogaster, and mammals that indicate major mysteries remaining to be solved...
  42. doi request reprint A chemical inhibitor of DRP1 uncouples mitochondrial fission and apoptosis
    Atsushi Tanaka
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20824, USA
    Mol Cell 29:409-10. 2008
    ..In a recent issue of Developmental Cell, Cassidy-Stone et al. (2008) identified mdivi-1, a new DRP1 inhibitor that prevents mitochondria division and Bax-mediated mitochondrial outer membrane permeabilization during apoptosis...
  43. ncbi request reprint Role of Bax and Bak in mitochondrial morphogenesis
    Mariusz Karbowski
    Biochemistry Section, SNB, NINDS, NIH, Bethesda, Maryland 20892, USA
    Nature 443:658-62. 2006
    ..Our results show that Bax and Bak regulate mitochondrial dynamics in healthy cells and indicate that Bcl-2 family members may also regulate apoptosis through organelle morphogenesis machineries...
  44. ncbi request reprint Scission, spores, and apoptosis: a proposal for the evolutionary origin of mitochondria in cell death induction
    Stephan Frank
    Biochemistry Section, SNB, NINDS NIH, Building 10, Room 5D 37, Bethesda, MD 20892, USA
    Biochem Biophys Res Commun 304:481-6. 2003
    ..This hypothesis would explain why what is generally considered the "power house" of the cell came to integrate the cell death response and regulate apoptosis...
  45. doi request reprint Role of the ubiquitin conjugation system in the maintenance of mitochondrial homeostasis
    Albert Neutzner
    Surgical Neurology Branch National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD, USA
    Ann N Y Acad Sci 1147:242-53. 2008
    ....
  46. doi request reprint Mitochondrial fission and fusion and their roles in the heart
    Lesley A Kane
    National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA
    J Mol Med (Berl) 88:971-9. 2010
    ..This review will provide an overview of mitochondrial fission and fusion as well as recent developments in the understanding of these processes in the heart...
  47. ncbi request reprint How do Bax and Bak lead to permeabilization of the outer mitochondrial membrane?
    Antonella Antignani
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 35 Convent Drive MSC 3704, Bethesda, MD 20892, USA
    Curr Opin Cell Biol 18:685-9. 2006
    ..Work linking Bcl-2 family members to mitochondrial morphogenesis in worms and mammals suggests some common functions of Bcl-2 family proteins may exist...
  48. pmc Cytomegalovirus proteins vMIA and m38.5 link mitochondrial morphogenesis to Bcl-2 family proteins
    Kristi L Norris
    Biochemistry Section, Surgical Neurology Branch, NINDS, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Virol 82:6232-43. 2008
    ..Thus, vMIA and m38.5 share some, but not all, features of apoptosis regulation through Bcl-2 family interaction and allow the dissection of Bax translocation into discrete steps...
  49. pmc Jak3-independent trafficking of the common gamma chain receptor subunit: chaperone function of Jaks revisited
    Sigrun R Hofmann
    National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, 10 Center Dr, Bldg 10, Rm 9N256, Bethesda, MD 20892 1820, USA
    Mol Cell Biol 24:5039-49. 2004
    ..However, full-length Jak3 is required for normal trafficking of this cytokine receptor/Jak pair, a finding that has important structural and clinical implications...
  50. ncbi request reprint The cytokine, granulocyte-macrophage colony-stimulating factor (GM-CSF), can deliver Bcl-XL as an extracellular fusion protein to protect cells from apoptosis and retain differentiation induction
    Antonella Antignani
    Biochemistry Section, Surgical Neurology Branch, NINDS, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 282:11246-54. 2007
    ..This fully human fusion protein has potential to prevent monocytopenia and represents a new strategy for engineering anti-apoptotic therapeutics...
  51. pmc Targeting mitochondrial dysfunction: role for PINK1 and Parkin in mitochondrial quality control
    Derek P Narendra
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
    Antioxid Redox Signal 14:1929-38. 2011
    ..This recent work suggests that Parkin and PINK1 may be among the first mammalian proteins identified with a direct role in regulating mitophagy, and implicate a failure of mitophagy in the pathogenesis of Parkinson's disease...
  52. pmc Mutations in Fis1 disrupt orderly disposal of defective mitochondria
    Qinfang Shen
    Department of Biological Chemistry, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095 Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 Department of Neurology, Juntendo University School of Medicine, Tokyo 113 8421, Japan
    Mol Biol Cell 25:145-59. 2014
    ..We conclude that Fis1 can act in sequence with Mff at the ER-mitochondrial interface to couple stress-induced mitochondrial fission with downstream degradation processes. ..
  53. ncbi request reprint Bcl-xL and caspase inhibition increase the survival of rat oxytocin and vasopressin magnocellular neurons in organotypic culture
    Shirley B House
    Laboratory of Neurochemistry, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Exp Neurol 200:267-71. 2006
    ..01) but not VP MCNs (P > 0.09). Unlike the Bcl-xL, Z-VAD-fmk's effectiveness in reducing MCN cell death was not sustained for the full 15 days in vitro...
  54. ncbi request reprint Morphology of mitochondria during apoptosis: worms-to-beetles in worms
    Richard J Youle
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20815, USA
    Dev Cell 8:298-9. 2005
    ..elegans development inhibits programmed cell death bridges this gap and should advance a more detailed understanding of the role of mitochondria in caspase activation...
  55. pmc Parkin is a lipid-responsive regulator of fat uptake in mice and mutant human cells
    Kye Young Kim
    Center for Molecular Medicine, NHLBI, 10 Center Drive, Bethesda, Maryland, 20892 1454, USA
    J Clin Invest 121:3701-12. 2011
    ..Whether this metabolic regulation contributes to premature Parkinsonism warrants investigation...
  56. ncbi request reprint Extracellular Bad fused to toxin transport domains induces apoptosis
    Makoto Ichinose
    Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
    Cancer Res 62:1433-8. 2002
    ..We conclude that extracellular Bad can be delivered into cells via the transport domain of a bacterial toxin and may be used to induce apoptosis...
  57. doi request reprint Involvement of mitochondrial dynamics in the segregation of mitochondrial matrix proteins during stationary phase mitophagy
    Hagai Abeliovich
    1 The Institute for Biochemistry, Food Science, and Nutrition, Robert H Smith Faculty of Agriculture, Food and Environment, Hebrew University of Jerusalem, P O Box 12, Rehovot, Israel 76100 2 Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, Porter Neuroscience Research Center Building 35, Room 2C 917 35 Convent Drive, Bethesda, Maryland 20892 3704, USA
    Nat Commun 4:2789. 2013
    ..In agreement, the rates of mitophagic degradation strongly correlate with the degree of physical segregation of specific matrix proteins. ..
  58. doi request reprint Mitochondrial fission and fusion
    Iain Scott
    National Heart, Lung and Blood Institute, National Institutes of Health, 9000 Rockville Pike, Bethesda, MD 20892, USA
    Essays Biochem 47:85-98. 2010
    ..In the present chapter we discuss the mechanisms behind mitochondrial fission and fusion, and discuss the implications of changes in organelle morphology during the life of a cell...
  59. ncbi request reprint PINK1 and Parkin Flag Miro to Direct Mitochondrial Traffic
    Lesley A Kane
    National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Cell 147:721-3. 2011
    ..In this issue of Cell, Wang et al. (2011) now show that PINK1 and Parkin also regulate mitochondrial trafficking and quarantine damaged mitochondria by severing their connection to the microtubule network...
  60. ncbi request reprint Entry into cells and selective degradation of tRNAs by a cytotoxic member of the RNase A family
    Shailendra K Saxena
    Biochemistry Section, Surgical Neurology Branch, NINDS, National Institutes of Health, Bethesda, Maryland 20892 1414, USA
    J Biol Chem 277:15142-6. 2002
    ..We conclude that the degradation of tRNAs may be a primary factor in the cytotoxic activity of onconase...
  61. pmc Nitric oxide-induced mitochondrial fission is regulated by dynamin-related GTPases in neurons
    Mark J Barsoum
    Apoptosis and Cell Death Program, Burnham Institute for Medical Research, La Jolla, CA, USA
    EMBO J 25:3900-11. 2006
    ..Importantly, NO-induced neuronal cell death was mitigated by Mfn1 and Drp1(K38A). Thus, persistent mitochondrial fission may play a causal role in NO-mediated neurotoxicity...
  62. ncbi request reprint The permeability transition pore signals apoptosis by directing Bax translocation and multimerization
    FrancesaA De Giorgi
    European Institute of Chemistry and Biology, and INSERM E 9929, Victor Segalen Bordeaux 2 University, 33076 Bordeaux Cedex, France
    FASEB J 16:607-9. 2002
    ..We conclude that the PTP is not itself a component of the Cyt.c release machinery, but that it acts indirectly by signaling Bax translocation and multimerization...
  63. pmc Loss of Bif-1 suppresses Bax/Bak conformational change and mitochondrial apoptosis
    Yoshinori Takahashi
    Drug Discovery Program, H Lee Moffitt Cancer Center and Research Institute, 12902 Magnolia Drive, Tampa, FL 33612, USA
    Mol Cell Biol 25:9369-82. 2005
    ..Taken together, these findings support the notion that Bif-1 is an important component of the mitochondrial pathway for apoptosis as a novel Bax/Bak activator, and loss of this proapoptotic molecule may contribute to tumorigenesis...
  64. ncbi request reprint Mitochondrial fission and fusion mediators, hFis1 and OPA1, modulate cellular senescence
    Seungmin Lee
    Department of Biochemistry, Ajou University School of Medicine, Ajou University, 5 Wonchon dong, Yeongtong Gu, Suwon 443 721, Korea
    J Biol Chem 282:22977-83. 2007
    ..Thus, one of the key functions of mitochondrial fission might be prevention of the sustained extensive mitochondrial elongation that triggers cellular senescence...
  65. ncbi request reprint Mitofusin-1 protein is a generally expressed mediator of mitochondrial fusion in mammalian cells
    Ansgar Santel
    Department of Developmental Biology, Stanford University School of Medicine, Stanford, CA 94305, USA
    J Cell Sci 116:2763-74. 2003
    ..Thus, Mfn1 appears to be a key player in mediating mitochondrial fusion and morphology in mammalian cells...
  66. ncbi request reprint JNK-mediated BIM phosphorylation potentiates BAX-dependent apoptosis
    Girish V Putcha
    Department of Neurology and Department of Molecular Biology and Pharmacology, Washington University School of Medicine, Saint Louis, MO 63110, USA
    Neuron 38:899-914. 2003
    ..Thus, JNKs regulate the proapoptotic activity of BIM(EL) during TFD, both transcriptionally and posttranslationally...
  67. pmc Role of mitochondrial remodeling in programmed cell death in Drosophila melanogaster
    Gaurav Goyal
    National Centre for Biological Sciences, Tata Institute of Fundamental Research, GKVK Campus, Bellary Road, Bangalore 560 065, India
    Dev Cell 12:807-16. 2007
    ..Thus, mitochondrial remodeling is capable of modifying the propensity of cells to undergo death in Drosophila...
  68. doi request reprint OPA1 mutations associated with dominant optic atrophy impair oxidative phosphorylation and mitochondrial fusion
    Claudia Zanna
    Dipartimento di Biologia Evoluzionistica Sperimentale, Universita di Bologna, Via Irnerio 42, 40126 Bologna, Italy
    Brain 131:352-67. 2008
    ..The results disclose a novel link between OPA1, apoptosis inducing factor and the respiratory complexes that may shed some light on the pathogenic mechanism of DOA...
  69. ncbi request reprint Drp-1-dependent division of the mitochondrial network blocks intraorganellar Ca2+ waves and protects against Ca2+-mediated apoptosis
    Gyorgy Szabadkai
    Department of Experimental and Diagnostic Medicine, Section of General Pathology and Interdisciplinary Center for the Study of Inflammation, University of Ferrara, 44100 Ferrara, Italy
    Mol Cell 16:59-68. 2004
    ....