{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T07:32:17Z","timestamp":1792222337591,"version":"4.4.0"},"reference-count":30,"publisher":"Springer Science and Business Media LLC","issue":"7498","license":[{"start":{"date-parts":[[2014,3,26]],"date-time":"2014-03-26T00:00:00Z","timestamp":1395792000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nature"],"published-print":{"date-parts":[[2014,5]]},"DOI":"10.1038\/nature13136","type":"journal-article","created":{"date-parts":[[2014,3,26]],"date-time":"2014-03-26T06:46:27Z","timestamp":1395816387000},"page":"96-100","source":"Crossref","is-referenced-by-count":397,"title":["Cystathionine \u03b3-lyase deficiency mediates neurodegeneration in Huntington\u2019s disease"],"prefix":"10.1038","volume":"509","author":[{"given":"Bindu D.","family":"Paul","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan I.","family":"Sbodio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Risheng","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. Scott","family":"Vandiver","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiyoung Y.","family":"Cha","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adele M.","family":"Snowman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Solomon H.","family":"Snyder","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2014,3,26]]},"reference":[{"issue":"6","key":"BFnature13136_CR1","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","volume":"72","author":"M MACDONALD","year":"1993","unstructured":"Huntington\u2019s Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington\u2019s disease chromosomes. Cell 72, 971\u2013983 (1993)","journal-title":"Cell"},{"key":"BFnature13136_CR2","doi-asserted-by":"publisher","first-page":"1187","DOI":"10.1016\/j.neuropharm.2010.10.025","volume":"60","author":"S Subramaniam","year":"2011","unstructured":"Subramaniam, S. & Snyder, S. H. Huntington\u2019s disease is a disorder of the corpus striatum: focus on Rhes (Ras homologue enriched in the striatum). Neuropharmacology 60, 1187\u20131192 (2011)","journal-title":"Neuropharmacology"},{"key":"BFnature13136_CR3","doi-asserted-by":"publisher","first-page":"499","DOI":"10.1038\/nrm3391","volume":"13","author":"BD Paul","year":"2012","unstructured":"Paul, B. D. & Snyder, S. H. H2S signalling through protein sulfhydration and beyond. Nature Rev. Mol. Cell Biol. 13, 499\u2013507 (2012)","journal-title":"Nature Rev. Mol. Cell Biol."},{"key":"BFnature13136_CR4","doi-asserted-by":"publisher","first-page":"791","DOI":"10.1152\/physrev.00017.2011","volume":"92","author":"R Wang","year":"2012","unstructured":"Wang, R. Physiological implications of hydrogen sulfide: a whiff exploration that blossomed. Physiol. Rev. 92, 791\u2013896 (2012)","journal-title":"Physiol. Rev."},{"key":"BFnature13136_CR5","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1007\/s00726-010-0510-x","volume":"41","author":"H Kimura","year":"2011","unstructured":"Kimura, H. Hydrogen sulfide: its production, release and functions. Amino Acids 41, 113\u2013121 (2011)","journal-title":"Amino Acids"},{"key":"BFnature13136_CR6","doi-asserted-by":"publisher","first-page":"1293","DOI":"10.1073\/pnas.1119658109","volume":"109","author":"T Morikawa","year":"2012","unstructured":"Morikawa, T. et al. Hypoxic regulation of the cerebral microcirculation is mediated by a carbon monoxide-sensitive hydrogen sulfide pathway. Proc. Natl Acad. Sci. USA 109, 1293\u20131298 (2012)","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"BFnature13136_CR7","doi-asserted-by":"publisher","first-page":"587","DOI":"10.1126\/science.1162667","volume":"322","author":"G Yang","year":"2008","unstructured":"Yang, G. et al. H2S as a physiologic vasorelaxant: hypertension in mice with deletion of cystathionine \u03b3-lyase. Science 322, 587\u2013590 (2008)","journal-title":"Science"},{"key":"BFnature13136_CR8","doi-asserted-by":"publisher","first-page":"493","DOI":"10.1016\/S0092-8674(00)81369-0","volume":"87","author":"L Mangiarini","year":"1996","unstructured":"Mangiarini, L. et al. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87, 493\u2013506 (1996)","journal-title":"Cell"},{"key":"BFnature13136_CR9","doi-asserted-by":"publisher","first-page":"e49838","DOI":"10.1371\/journal.pone.0049838","volume":"7","author":"LB Menalled","year":"2012","unstructured":"Menalled, L. B. et al. Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington\u2019s disease: zQ175. PLoS ONE 7, e49838 (2012)","journal-title":"PLoS ONE"},{"key":"BFnature13136_CR10","doi-asserted-by":"publisher","first-page":"26358","DOI":"10.1074\/jbc.M110.147439","volume":"285","author":"I Ishii","year":"2010","unstructured":"Ishii, I. et al. Cystathionine \u03b3-lyase-deficient mice require dietary cysteine to protect against acute lethal myopathy and oxidative injury. J. Biol. Chem. 285, 26358\u201326368 (2010)","journal-title":"J. Biol. Chem."},{"key":"BFnature13136_CR11","doi-asserted-by":"publisher","first-page":"1280","DOI":"10.1016\/j.freeradbiomed.2011.01.038","volume":"50","author":"S Mani","year":"2011","unstructured":"Mani, S., Yang, G. & Wang, R. A critical life-supporting role for cystathionine \u03b3-lyase in the absence of dietary cysteine supply. Free Radic. Biol. Med. 50, 1280\u20131287 (2011)","journal-title":"Free Radic. Biol. Med."},{"key":"BFnature13136_CR12","doi-asserted-by":"publisher","first-page":"1990","DOI":"10.1126\/science.277.5334.1990","volume":"277","author":"M DiFiglia","year":"1997","unstructured":"DiFiglia, M. et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277, 1990\u20131993 (1997)","journal-title":"Science"},{"key":"BFnature13136_CR13","doi-asserted-by":"publisher","first-page":"537","DOI":"10.1016\/S0092-8674(00)80513-9","volume":"90","author":"SW Davies","year":"1997","unstructured":"Davies, S. W. et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90, 537\u2013548 (1997)","journal-title":"Cell"},{"key":"BFnature13136_CR14","doi-asserted-by":"publisher","first-page":"233","DOI":"10.1016\/S0168-9525(03)00074-X","volume":"19","author":"KL Sugars","year":"2003","unstructured":"Sugars, K. L. & Rubinsztein, D. C. Transcriptional abnormalities in Huntington disease. Trends Genet. 19, 233\u2013238 (2003)","journal-title":"Trends Genet."},{"key":"BFnature13136_CR15","doi-asserted-by":"publisher","first-page":"1241","DOI":"10.1016\/j.cell.2005.10.030","volume":"123","author":"W Zhai","year":"2005","unstructured":"Zhai, W., Jeong, H., Cui, L., Krainc, D. & Tjian, R. In vitro analysis of huntingtin-mediated transcriptional repression reveals multiple transcription factor targets. Cell 123, 1241\u20131253 (2005)","journal-title":"Cell"},{"key":"BFnature13136_CR16","doi-asserted-by":"publisher","first-page":"2238","DOI":"10.1126\/science.1072613","volume":"296","author":"AW Dunah","year":"2002","unstructured":"Dunah, A. W. et al. Sp1 and TAFII130 transcriptional activity is disrupted in early Huntington\u2019s disease. Science 296, 2238\u20132243 (2002)","journal-title":"Science"},{"key":"BFnature13136_CR17","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1042\/BJ20040243","volume":"381","author":"I Ishii","year":"2004","unstructured":"Ishii, I. et al. Murine cystathionine \u03b3-lyase: complete cDNA and genomic sequences, promoter activity, tissue distribution and developmental expression. Biochem. J. 381, 113\u2013123 (2004)","journal-title":"Biochem. J."},{"key":"BFnature13136_CR18","doi-asserted-by":"publisher","first-page":"26450","DOI":"10.1074\/jbc.M111.266643","volume":"286","author":"G Yang","year":"2011","unstructured":"Yang, G., Pei, Y., Teng, H., Cao, Q. & Wang, R. Specificity protein-1 as a critical regulator of human cystathionine \u03b3-lyase in smooth muscle cells. J. Biol. Chem. 286, 26450\u201326460 (2011)","journal-title":"J. Biol. Chem."},{"key":"BFnature13136_CR19","doi-asserted-by":"publisher","first-page":"787","DOI":"10.1038\/nature05292","volume":"443","author":"MT Lin","year":"2006","unstructured":"Lin, M. T. & Beal, M. F. Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases. Nature 443, 787\u2013795 (2006)","journal-title":"Nature"},{"key":"BFnature13136_CR20","doi-asserted-by":"publisher","first-page":"2943","DOI":"10.1073\/pnas.1115634109","volume":"109","author":"M Fu","year":"2012","unstructured":"Fu, M. et al. Hydrogen sulfide (H2S) metabolism in mitochondria and its regulatory role in energy production. Proc. Natl Acad. Sci. USA 109, 2943\u20132948 (2012)","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"BFnature13136_CR21","doi-asserted-by":"publisher","first-page":"3767","DOI":"10.1073\/pnas.74.9.3767","volume":"74","author":"TA Alston","year":"1977","unstructured":"Alston, T. A., Mela, L. & Bright, H. J. 3-Nitropropionate, the toxic substance of Indigofera, is a suicide inactivator of succinate dehydrogenase. Proc. Natl Acad. Sci. USA 74, 3767\u20133771 (1977)","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"BFnature13136_CR22","doi-asserted-by":"publisher","first-page":"1521","DOI":"10.1111\/j.1471-4159.2005.03515.x","volume":"95","author":"E Brouillet","year":"2005","unstructured":"Brouillet, E., Jacquard, C., Bizat, N. & Blum, D. 3-Nitropropionic acid: a mitochondrial toxin to uncover physiopathological mechanisms underlying striatal degeneration in Huntington\u2019s disease. J. Neurochem. 95, 1521\u20131540 (2005)","journal-title":"J. Neurochem."},{"key":"BFnature13136_CR23","first-page":"ra72","volume":"2","author":"AK Mustafa","year":"2009","unstructured":"Mustafa, A. K. et al. H2S signals through protein S-sulfhydration. Sci. Signal. 2, ra72 (2009)","journal-title":"Sci. Signal."},{"key":"BFnature13136_CR24","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1016\/j.molcel.2011.10.021","volume":"45","author":"N Sen","year":"2012","unstructured":"Sen, N. et al. Hydrogen sulfide-linked sulfhydration of NF-\u03baB mediates its antiapoptotic actions. Mol. Cell 45, 13\u201324 (2012)","journal-title":"Mol. Cell"},{"key":"BFnature13136_CR25","doi-asserted-by":"publisher","first-page":"1626","DOI":"10.1038\/ncomms2623","volume":"4","author":"MS Vandiver","year":"2013","unstructured":"Vandiver, M. S. et al. Sulfhydration mediates neuroprotective actions of parkin. Nature. Commun. 4, 1626 (2013)","journal-title":"Nature. Commun."},{"key":"BFnature13136_CR26","doi-asserted-by":"publisher","first-page":"1906","DOI":"10.1089\/ars.2012.4645","volume":"18","author":"G Yang","year":"2013","unstructured":"Yang, G. et al. Hydrogen sulfide protects against cellular senescence via S-sulfhydration of Keap1 and activation of Nrf2. Antioxid. Redox Signal. 18, 1906\u20131919 (2013)","journal-title":"Antioxid. Redox Signal."},{"key":"BFnature13136_CR27","doi-asserted-by":"publisher","first-page":"917","DOI":"10.1038\/nrd2425","volume":"6","author":"C Szabo","year":"2007","unstructured":"Szabo, C. Hydrogen sulphide and its therapeutic potential. Nature Rev. Drug Discov. 6, 917\u2013935 (2007)","journal-title":"Nature Rev. Drug Discov."},{"key":"BFnature13136_CR28","doi-asserted-by":"publisher","first-page":"2355","DOI":"10.1098\/rstb.2005.1770","volume":"360","author":"W Droge","year":"2005","unstructured":"Droge, W. Oxidative stress and ageing: is ageing a cysteine deficiency syndrome? Phil. Trans. R. Soc. Lond. B 360, 2355\u20132372 (2005)","journal-title":"Phil. Trans. R. Soc. Lond. B"},{"key":"BFnature13136_CR29","doi-asserted-by":"publisher","first-page":"413","DOI":"10.1111\/j.1471-4159.2004.02726.x","volume":"91","author":"JH Fox","year":"2004","unstructured":"Fox, J. H. et al. Cystamine increases l-cysteine levels in Huntington\u2019s disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation. J. Neurochem. 91, 413\u2013422 (2004)","journal-title":"J. Neurochem."},{"key":"BFnature13136_CR30","doi-asserted-by":"publisher","first-page":"83","DOI":"10.1016\/j.neuroimage.2005.01.039","volume":"26","author":"M Cyr","year":"2005","unstructured":"Cyr, M., Caron, M. G., Johnson, G. A. & Laakso, A. Magnetic resonance imaging at microscopic resolution reveals subtle morphological changes in a mouse model of dopaminergic hyperfunction. Neuroimage 26, 83\u201390 (2005)","journal-title":"Neuroimage"}],"container-title":["Nature"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.nature.com\/articles\/nature13136.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.nature.com\/articles\/nature13136","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.nature.com\/articles\/nature13136.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,18]],"date-time":"2023-05-18T14:19:55Z","timestamp":1684419595000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/nature13136"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,3,26]]},"references-count":30,"journal-issue":{"issue":"7498","published-print":{"date-parts":[[2014,5]]}},"alternative-id":["BFnature13136"],"URL":"https:\/\/doi.org\/10.1038\/nature13136","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.718326502.793493986","asserted-by":"object"}]},"ISSN":["0028-0836","1476-4687"],"issn-type":[{"value":"0028-0836","type":"print"},{"value":"1476-4687","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,3,26]]}}}