دورية أكاديمية
أعرض في EDS

Inhibition of Drp1/Fis1 interaction slows progression of amyotrophic lateral sclerosis.

التفاصيل البيبلوغرافية
العنوان: Inhibition of Drp1/Fis1 interaction slows progression of amyotrophic lateral sclerosis.
المؤلفون: Joshi AU; Department of Chemical and Systems Biology, Stanford University School of Medicine, Stanford, CA, USA., Saw NL; Behavioral and Functional Neuroscience Laboratory, Department of Neurosurgery, Stanford University School of Medicine, Stanford, CA, USA., Vogel H; Department of Pathology, Stanford University School of Medicine, Stanford, CA, USA., Cunnigham AD; Department of Chemical and Systems Biology, Stanford University School of Medicine, Stanford, CA, USA., Shamloo M; Behavioral and Functional Neuroscience Laboratory, Department of Neurosurgery, Stanford University School of Medicine, Stanford, CA, USA., Mochly-Rosen D; Department of Chemical and Systems Biology, Stanford University School of Medicine, Stanford, CA, USA mochly@stanford.edu.
المصدر: EMBO molecular medicine [EMBO Mol Med] 2018 Mar; Vol. 10 (3).
نوع المنشور: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
اللغة: English
بيانات الدورية: Publisher: EMBO Press Country of Publication: England NLM ID: 101487380 Publication Model: Print Cited Medium: Internet ISSN: 1757-4684 (Electronic) Linking ISSN: 17574676 NLM ISO Abbreviation: EMBO Mol Med Subsets: MEDLINE
أسماء مطبوعة: Publication: 2024- : Heidelberg : EMBO Press
Original Publication: Chichester, West Sussex : Wiley-Blackwell
مواضيع طبية MeSH: Disease Progression*, Amyotrophic Lateral Sclerosis/*metabolism , Amyotrophic Lateral Sclerosis/*pathology , GTP Phosphohydrolases/*metabolism , Membrane Proteins/*metabolism , Microtubule-Associated Proteins/*metabolism , Mitochondrial Proteins/*metabolism, Animals ; Apoptosis/drug effects ; Autophagy/drug effects ; Behavior, Animal ; Cell Differentiation/drug effects ; Disease Models, Animal ; Dynamins ; Fibroblasts/drug effects ; Fibroblasts/metabolism ; Fibroblasts/pathology ; GTP Phosphohydrolases/pharmacology ; Humans ; Mice ; Mitochondria/drug effects ; Mitochondria/metabolism ; Mitochondria/ultrastructure ; Mitochondrial Dynamics/drug effects ; Models, Biological ; Motor Activity/drug effects ; Motor Neurons/drug effects ; Motor Neurons/metabolism ; Motor Neurons/pathology ; Muscular Atrophy/pathology ; Mutation/genetics ; Oxidative Stress/drug effects ; Peptide Fragments/pharmacology ; Protein Binding/drug effects ; Stress, Physiological/drug effects ; Superoxide Dismutase/metabolism
مستخلص: Bioenergetic failure and oxidative stress are common pathological hallmarks of amyotrophic lateral sclerosis (ALS), but whether these could be targeted effectively for novel therapeutic intervention needs to be determined. One of the reported contributors to ALS pathology is mitochondrial dysfunction associated with excessive mitochondrial fission and fragmentation, which is predominantly mediated by Drp1 hyperactivation. Here, we determined whether inhibition of excessive fission by inhibiting Drp1/Fis1 interaction affects disease progression. We observed mitochondrial excessive fragmentation and dysfunction in several familial forms of ALS patient-derived fibroblasts as well as in cultured motor neurons expressing SOD1 mutant. In both cell models, inhibition of Drp1/Fis1 interaction by a selective peptide inhibitor, P110, led to a significant reduction in reactive oxygen species levels, and to improvement in mitochondrial structure and functions. Sustained treatment of mice expressing G93A SOD1 mutation with P110, beginning at the onset of disease symptoms at day 90, produced an improvement in motor performance and survival, suggesting that Drp1 hyperactivation may be an attractive target in the treatment of ALS patients.
(© 2018 The Authors. Published under the terms of the CC BY 4.0 license.)
References: J Neurochem. 2003 Jul;86(2):363-73. (PMID: 12871577)
Lancet Neurol. 2017 Oct;16(10 ):772. (PMID: 28920880)
Biochim Biophys Acta. 2006 Nov-Dec;1762(11-12):1038-50. (PMID: 16876390)
Amyotroph Lateral Scler Frontotemporal Degener. 2017 Oct;18(sup1):98-103. (PMID: 28872911)
Cell Death Discov. 2015 Sep 14;1:15030. (PMID: 27551461)
Brain. 2015 Apr;138(Pt 4):875-90. (PMID: 25678561)
EMBO Rep. 2006 Sep;7(9):880-5. (PMID: 16953201)
Aging (Albany NY). 2017 Mar 26;9(3):627-649. (PMID: 28351997)
J Neurosci Methods. 2016 Mar 15;262:56-65. (PMID: 26777473)
J Neurochem. 2005 Jul;94(1):192-203. (PMID: 15953362)
J Cell Sci. 2013 Feb 1;126(Pt 3):789-802. (PMID: 23239023)
Acta Neuropathol Commun. 2016 May 05;4(1):47. (PMID: 27151080)
Brain. 2002 Jul;125(Pt 7):1522-33. (PMID: 12077002)
Autophagy. 2016 Jul 2;12 (7):1094-104. (PMID: 27158844)
Acta Neuropathol Commun. 2016 Apr 27;4(1):43. (PMID: 27121871)
Amyotroph Lateral Scler Frontotemporal Degener. 2017 Oct;18(sup1):88-97. (PMID: 28872912)
PLoS One. 2013 Dec 06;8(12):e82112. (PMID: 24324755)
BMC Pulm Med. 2016 Nov 3;16(1):136. (PMID: 27809826)
J Vis Exp. 2014 Sep 29;(91):51785. (PMID: 25286313)
Methods Mol Biol. 2015;1292:97-104. (PMID: 25804750)
Folia Neuropathol. 2004;42(3):151-6. (PMID: 15535033)
PLoS One. 2011;6(7):e22031. (PMID: 21779368)
Autophagy. 2010 Nov;6(8):1090-106. (PMID: 20890124)
J Clin Invest. 2008 Jan;118(1):173-82. (PMID: 18097471)
Physiol Rep. 2016 Aug;4(15):. (PMID: 27511983)
Hum Mol Genet. 2007 Nov 15;16(22):2720-2728. (PMID: 17725983)
Expert Opin Pharmacother. 2017 May;18(7):735-738. (PMID: 28406335)
Hum Mol Genet. 2014 Mar 15;23 (6):1413-24. (PMID: 24154542)
Dev Cell. 2001 Oct;1(4):515-25. (PMID: 11703942)
J Neurol. 2015 Jun;262(6):1493-503. (PMID: 25893255)
Mol Brain. 2017 Jun 13;10 (1):22. (PMID: 28610619)
Hum Mol Genet. 2010 Oct 15;19(20):3919-35. (PMID: 20660112)
FEBS J. 2011 Apr;278(6):941-54. (PMID: 21232014)
J Biol Chem. 2007 Apr 13;282(15):11521-9. (PMID: 17301055)
J Biol Chem. 2007 Apr 13;282(15):11068-77. (PMID: 17296612)
PLoS One. 2011;6(12):e28536. (PMID: 22194845)
Neuron. 2006 Oct 5;52(1):39-59. (PMID: 17015226)
FEBS Lett. 2003 Sep 11;551(1-3):104-6. (PMID: 12965212)
Circ Res. 2015 May 22;116(11):1835-49. (PMID: 25999423)
Mol Neurobiol. 2014 Jun;49(3):1435-48. (PMID: 24390572)
Curr Neurovasc Res. 2013 Aug;10(3):222-30. (PMID: 23713734)
Lancet Neurol. 2010 Oct;9(10):995-1007. (PMID: 20864052)
Biochim Biophys Acta. 2006 Nov-Dec;1762(11-12):1051-67. (PMID: 16713195)
J Neurol Neurosurg Psychiatry. 2014 Nov;85(11):1232-8. (PMID: 24648037)
Neurochem Res. 2016 May;41(5):965-84. (PMID: 26646005)
Neurobiol Dis. 2014 Apr;64:48-59. (PMID: 24361555)
Proc Natl Acad Sci U S A. 2017 Sep 26;114(39):E8294-E8303. (PMID: 28904095)
Neurobiol Dis. 2016 Jun;90:35-42. (PMID: 26282323)
J Bioenerg Biomembr. 2011 Dec;43(6):587-92. (PMID: 22072073)
Amyotroph Lateral Scler. 2012 Feb;13(2):166-77. (PMID: 22292840)
Mol Cell Pharmacol. 2013;5(3):109-133. (PMID: 24533171)
J Cell Sci. 2016 Jun 1;129(11):2170-81. (PMID: 27076521)
Neuron. 2016 Oct 19;92 (2):383-391. (PMID: 27720481)
Mol Brain. 2017 Feb 2;10 (1):5. (PMID: 28148298)
Dev Cell. 2008 Feb;14(2):193-204. (PMID: 18267088)
Lancet. 1996 May 25;347(9013):1425-31. (PMID: 8676624)
J Am Heart Assoc. 2013 Oct 08;2(5):e000461. (PMID: 24103571)
Biochem Biophys Res Commun. 2005 Jul 29;333(2):650-9. (PMID: 15979461)
Exp Neurol. 2008 Oct;213(2):448-55. (PMID: 18718468)
EMBO Rep. 2010 Sep;11(9):678-84. (PMID: 20725092)
J Neurochem. 2002 Feb;80(4):616-25. (PMID: 11841569)
Arch Clin Neuropsychol. 2017 Nov 1;32(7):906-916. (PMID: 29028904)
Biochem Biophys Res Commun. 2009 Sep 11;387(1):202-6. (PMID: 19580782)
Prog Neurobiol. 2008 May;85(1):94-134. (PMID: 18282652)
J Clin Invest. 2013 Dec;123(12):5371-88. (PMID: 24231356)
Mol Cell Biol. 2004 Sep;24(18):8055-68. (PMID: 15340068)
Muscle Nerve. 2006 Jun;33(6):809-16. (PMID: 16583367)
Nat Med. 2016 Aug;22(8):869-78. (PMID: 27348499)
Biochim Biophys Acta. 2015 Nov;1852(11):2517-24. (PMID: 26264610)
Lancet Neurol. 2017 Jul;16(7):490-491. (PMID: 28522180)
Behav Brain Res. 2012 Jul 15;233(1):55-61. (PMID: 22569582)
Cell Mol Neurobiol. 2014 Apr;34(3):451-62. (PMID: 24442855)
Front Mol Neurosci. 2014 Jul 31;7:70. (PMID: 25132814)
Amyotroph Lateral Scler. 2012 Jun;13(4):367-71. (PMID: 22632443)
J Biol Chem. 2009 May 15;284(20):13843-55. (PMID: 19279012)
Prog Neurobiol. 2012 May;97(2):54-66. (PMID: 21827820)
Hum Mol Genet. 2009 Jan 1;18(1):82-96. (PMID: 18826962)
J Neurosci. 2004 Aug 4;24(31):6880-8. (PMID: 15295022)
Biochim Biophys Acta. 2014 Aug;1842(8):1295-301. (PMID: 24568860)
Front Cell Neurosci. 2015 Aug 25;9:336. (PMID: 26379505)
Dev Cell. 2017 Mar 27;40(6):583-594.e6. (PMID: 28350990)
Science. 1994 Jun 17;264(5166):1772-5. (PMID: 8209258)
J Exp Med. 2016 Nov 14;213(12 ):2655-2669. (PMID: 27821553)
Cell Metab. 2008 Nov;8(5):425-36. (PMID: 19046573)
Front Cell Neurosci. 2015 May 18;9:170. (PMID: 26041991)
Nat Rev Neurol. 2015 Jun;11(6):360-6. (PMID: 25939274)
Mol Biol Cell. 2013 Mar;24(5):659-67. (PMID: 23283981)
Cell Death Differ. 2013 Dec;20(12 ):1615-30. (PMID: 24096871)
Science. 2012 Aug 31;337(6098):1062-5. (PMID: 22936770)
J Cell Sci. 2014 Mar 15;127(Pt 6):1263-78. (PMID: 24424030)
Neuron. 2016 Jul 6;91(1):41-55. (PMID: 27321923)
Physiol Rev. 2009 Jul;89(3):799-845. (PMID: 19584314)
Lancet Neurol. 2017 Jul;16(7):505-512. (PMID: 28522181)
Cell Rep. 2017 Mar 14;18(11):2592-2599. (PMID: 28297664)
Dev Neurobiol. 2017 Nov;77(11):1260-1268. (PMID: 28842943)
Neurobiol Dis. 2013 Mar;51:72-81. (PMID: 22819776)
Biochim Biophys Acta. 2008 Dec;1782(12):691-9. (PMID: 18930136)
معلومات مُعتمدة: P30 NS069375 United States NS NINDS NIH HHS; R37 AA011147 United States AA NIAAA NIH HHS
فهرسة مساهمة: Keywords: Protein–Protein interactions; amyotrophic lateral sclerosis; dynamin‐related protein 1; fission 1; mitochondrial dysfunction
المشرفين على المادة: 0 (FIS1 protein, human)
0 (Membrane Proteins)
0 (Microtubule-Associated Proteins)
0 (Mitochondrial Proteins)
0 (P110 peptide)
0 (Peptide Fragments)
EC 1.15.1.1 (Superoxide Dismutase)
EC 3.6.1.- (GTP Phosphohydrolases)
EC 3.6.5.5 (DNM1L protein, human)
EC 3.6.5.5 (Dynamins)
تواريخ الأحداث: Date Created: 20180117 Date Completed: 20190204 Latest Revision: 20201209
رمز التحديث: 20240628
مُعرف محوري في PubMed: PMC5840540
DOI: 10.15252/emmm.201708166
PMID: 29335339
قاعدة البيانات: MEDLINE
الوصف
تدمد:1757-4684
DOI:10.15252/emmm.201708166