{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,5]],"date-time":"2025-10-05T19:41:14Z","timestamp":1759693274856,"version":"build-2065373602"},"reference-count":50,"publisher":"Elsevier BV","issue":"5","license":[{"start":{"date-parts":[[1999,5,1]],"date-time":"1999-05-01T00:00:00Z","timestamp":925516800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[1999,5,1]],"date-time":"1999-05-01T00:00:00Z","timestamp":925516800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2003,5,25]],"date-time":"2003-05-25T00:00:00Z","timestamp":1053820800000},"content-version":"vor","delay-in-days":1485,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Medical Hypotheses"],"published-print":{"date-parts":[[1999,5]]},"DOI":"10.1054\/mehy.1998.0771","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T07:36:10Z","timestamp":1027668970000},"page":"377-382","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":5,"title":["Neurodegeneration in ataxia\u2013telangiectasia is caused by horror autotoxicus"],"prefix":"10.1054","volume":"52","author":[{"given":"R.O.","family":"Kuljis","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.A.","family":"Aguila","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1054\/mehy.1998.0771_RF1","first-page":"33","article-title":"Sur un syndrome progressif comprenant des t\u00e9langiectasies capillaires cutan\u00e9es et conjonctivales sym\u00e9triques, \u00e0 disposition naevo\u0131&#x0308;de, et des troubles c\u00e9r\u00e9belleux","volume":"4","author":"Louis-Bar","year":"1941","journal-title":"Confinia Neurol"},{"key":"10.1054\/mehy.1998.0771_RF2","first-page":"541","article-title":"Contribution a l'ind\u00e9pendance de l\u2019at\u00e9those double idiopathique et cong\u00e9nitale: atteinte familiale, syndrome dystrophique, signe du r\u00e9seau vasculaire conjonctival, int\u00e9grit\u00e9 psyquique","volume":"1","author":"Syllaba","year":"1926","journal-title":"Rev Neurol"},{"key":"10.1054\/mehy.1998.0771_RF3","first-page":"255","article-title":"Ataxia-telangiectasia: some historic, clinical and pathological observations","volume":"11","author":"Boder","year":"1975","journal-title":"Birth Defects"},{"key":"10.1054\/mehy.1998.0771_RF4","doi-asserted-by":"crossref","first-page":"S23","DOI":"10.1080\/09553009414551821","article-title":"Clinical and genetic features of ataxia-telangiectasia","volume":"66","author":"Bundey","year":"1994","journal-title":"Int J Radiat Biol"},{"key":"10.1054\/mehy.1998.0771_RF5","series-title":"Handbook of Clinical Neurology","first-page":"347","article-title":"Ataxia-telangiectasia","author":"Sedgwick","year":"1991"},{"key":"10.1054\/mehy.1998.0771_RF6","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1097\/00005072-196810000-00009","article-title":"Pathological observations in ataxia-telangiectasia. A report on five cases","volume":"27","author":"Aguilar","year":"1968","journal-title":"J Neuropathol Exp Neurol"},{"key":"10.1054\/mehy.1998.0771_RF7","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1136\/jnnp.29.6.489","article-title":"Pathological findings in three cases of ataxia\u2013telangiectasia","volume":"29","author":"Strich","year":"1966","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"10.1054\/mehy.1998.0771_RF8","doi-asserted-by":"crossref","first-page":"1749","DOI":"10.1126\/science.7792600","article-title":"A single ataxia-telangiectasia gene with a product similar to PI-3 kinase","volume":"268","author":"Savitsky","year":"1995","journal-title":"Science"},{"key":"10.1054\/mehy.1998.0771_RF9","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1007\/BF01541389","article-title":"Ataxia-telangiectasia and the ATM gene: linking neurodegeneration, immunodeficiency, and cancer cycle checkpoints","volume":"16","author":"Shiloh","year":"1996","journal-title":"J Clin Immunol"},{"key":"10.1054\/mehy.1998.0771_RF10","doi-asserted-by":"crossref","first-page":"2025","DOI":"10.1093\/hmg\/4.11.2025","article-title":"The complete sequence of the coding region of the ATM gene reveals similarity to cell cycle regulators in different species","volume":"4","author":"Savitsky","year":"1995","journal-title":"Hum Mol Genet"},{"key":"10.1054\/mehy.1998.0771_RF11","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1056\/NEJM199509073331014","article-title":"Ataxia-telangiectasia: broad implications for a rare disorder","volume":"333","author":"Kastan","year":"1995","journal-title":"N Engl J Med"},{"key":"10.1054\/mehy.1998.0771_RF12","doi-asserted-by":"crossref","first-page":"516","DOI":"10.1038\/387516a0","article-title":"Ataxia telangiectasia mutant protein activates c-abl tyrosine kinase in response to ionizing radiation","volume":"387","author":"Baskaran","year":"1997","journal-title":"Nature"},{"key":"10.1054\/mehy.1998.0771_RF13","doi-asserted-by":"crossref","first-page":"2411","DOI":"10.1101\/gad.10.19.2411","article-title":"Targeted disruption of ATM leads to growth retardation, chromosomal fragmentation during meiosis, immune defects, and thymic lymphoma","volume":"10","author":"Xu","year":"1996","journal-title":"Genes Dev"},{"key":"10.1054\/mehy.1998.0771_RF14","doi-asserted-by":"crossref","first-page":"13084","DOI":"10.1073\/pnas.93.23.13084","article-title":"Pleiotropic defects in ataxia-telangiectasia protein-deficiency mice","volume":"93","author":"Elson","year":"1996","journal-title":"Proc Natl Acad Sci"},{"key":"10.1054\/mehy.1998.0771_RF15","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1016\/S0092-8674(00)80086-0","article-title":"Atm-deficient mice: a paradigm of ataxia-telangiectasia","volume":"86","author":"Barlow","year":"1996","journal-title":"Cell"},{"key":"10.1054\/mehy.1998.0771_RF16","doi-asserted-by":"crossref","first-page":"12688","DOI":"10.1073\/pnas.94.23.12688","article-title":"Degeneration of neurons, synapses and neuropil, and glial activation, in a murine Atm knockout model of ataxia-telangiectasia","volume":"94","author":"Kuljis","year":"1997","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1054\/mehy.1998.0771_RF17","doi-asserted-by":"crossref","first-page":"1931","DOI":"10.1212\/WNL.42.10.1931","article-title":"Paraneoplastic cerebellar degeneration. I. A clinical analysis of 55 anti-Yo antibody positive patients","volume":"42","author":"Peterson","year":"1992","journal-title":"Neurology"},{"year":"1995","series-title":"Neurologic Complications of Cancer","author":"Posner","key":"10.1054\/mehy.1998.0771_RF18"},{"key":"10.1054\/mehy.1998.0771_RF19","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1097\/00005792-198803000-00003","article-title":"Opsoclonus, myoclonus, ataxia and encephalopathy in adults with cancer: a distinct paraneoplastic syndrome","volume":"67","author":"Anderson","year":"1988","journal-title":"Medicine"},{"key":"10.1054\/mehy.1998.0771_RF20","doi-asserted-by":"crossref","first-page":"S102","DOI":"10.1002\/ana.410370711","article-title":"Autoimmune central nervous system paraneoplastic disorders: mechanisms, diagnosis, and therapeutic options","volume":"37","author":"Dropcho","year":"1995","journal-title":"Ann Neurol"},{"key":"10.1054\/mehy.1998.0771_RF21","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1001\/archneur.1978.00500330001001","article-title":"Ataxia telangiectasia","volume":"35","author":"Teplitz","year":"1978","journal-title":"Arch Neurol"},{"key":"10.1054\/mehy.1998.0771_RF22","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1016\/S0022-3476(71)80353-0","article-title":"Autoimmune phenomena in ataxia telangiectasia","volume":"78","author":"Ammann","year":"1971","journal-title":"J Pediatr"},{"key":"10.1054\/mehy.1998.0771_RF23","first-page":"375","article-title":"Autoimmunity in the relatives of patients with immunodeficiency diseases","volume":"28","author":"Friedman","year":"1977","journal-title":"Clin Exp Immunol"},{"key":"10.1054\/mehy.1998.0771_RF24","first-page":"29","article-title":"Ataxia telangectasia. Probability of damaging immune mechanisms","volume":"34","author":"Berron","year":"1977","journal-title":"Bol Med Hosp Infant Mex"},{"key":"10.1054\/mehy.1998.0771_RF25","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1007\/BF00466267","article-title":"Immunological investigations in two brothers with ataxia telangiectasia Louis\u2013Bar","volume":"122","author":"Schulte-Wisserman","year":"1976","journal-title":"Eur J Pediatr"},{"key":"10.1054\/mehy.1998.0771_RF26","doi-asserted-by":"crossref","first-page":"867","DOI":"10.1016\/S0950-351X(05)80171-5","article-title":"Diabetes secondary to genetic disorders","volume":"6","author":"Robinson","year":"1992","journal-title":"Baillire's Clin Endocrinol Metab"},{"key":"10.1054\/mehy.1998.0771_RF27","doi-asserted-by":"crossref","first-page":"1164","DOI":"10.1056\/NEJM197805252982103","article-title":"Extreme insulin resistance in ataxia telangiectasia: defect in affinity of insulin receptors","volume":"298","author":"Bar","year":"1978","journal-title":"N Engl J Med"},{"key":"10.1054\/mehy.1998.0771_RF28","first-page":"149","article-title":"The nude mouse: a model of deficient T-cell function","volume":"7","author":"Pelleitier","year":"1975","journal-title":"Methods Achiev Exp Pathol"},{"key":"10.1054\/mehy.1998.0771_RF29","doi-asserted-by":"crossref","first-page":"324","DOI":"10.1097\/00005072-196804000-00009","article-title":"Ataxia in the neonatally thymectomized rat: a morphologic study of the central nervous system","volume":"27","author":"Tedeschi","year":"1968","journal-title":"J Neuropathol Exp Neurol"},{"key":"10.1054\/mehy.1998.0771_RF30","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1016\/0090-1229(77)90056-3","article-title":"Ataxia telangiectasia: an autoimmune disease associated with a cytotoxic antibody to brain and thymus","volume":"7","author":"Kaufman","year":"1977","journal-title":"Clin Immunol Immunopathol"},{"key":"10.1054\/mehy.1998.0771_RF31","first-page":"75","article-title":"Dysfunction of immune system and induction of autoantibodies to liver antigens by neonatal thymectomy in mice","volume":"45","author":"Myozaki","year":"1996","journal-title":"Hiroshima J Med Sci"},{"key":"10.1054\/mehy.1998.0771_RF32","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1097\/00005072-197909000-00003","article-title":"Ataxia-telangiectasia. Report of a case with Lewy bodies and vascular abnormalities within cerebral tissue","volume":"38","author":"Agamanolis","year":"1979","journal-title":"J Neuropathol Exp Neurol"},{"key":"10.1054\/mehy.1998.0771_RF33","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1016\/0006-8993(84)90118-5","article-title":"Mammalian brain antigens defined by monoclonal antibodies","volume":"322","author":"De Blas","year":"1984","journal-title":"Brain Res"},{"key":"10.1054\/mehy.1998.0771_RF34","doi-asserted-by":"crossref","first-page":"1777","DOI":"10.1212\/WNL.46.6.1777","article-title":"Anti-Yo antibody binding to 62-, 52-, or 58-kd protein recognizes a single molecule","volume":"46","author":"Tanaka","year":"1996","journal-title":"Neurology"},{"key":"10.1054\/mehy.1998.0771_RF35","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/0303-8467(95)00006-6","volume":"97","author":"Tanaka","year":"1995","journal-title":"Clin Neurol Neurosurg"},{"key":"10.1054\/mehy.1998.0771_RF36","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/0304-3940(95)11894-3","article-title":"Expression of messenger RNA encoding a paraneoplastic cerebellar degeneration-associated antigen in the rat hippocampus","volume":"197","author":"Sakai","year":"1995","journal-title":"Neurosci Lett"},{"key":"10.1054\/mehy.1998.0771_RF37","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/0165-5728(95)00063-8","article-title":"Induction of anti-Purkinje cell antibodies in vivo by immunizing with a recombinant 52-kDa paraneoplastic cerebellar degeneration-associated protein","volume":"60","author":"Sakai","year":"1995","journal-title":"J Neuroimmunol"},{"key":"10.1054\/mehy.1998.0771_RF38","doi-asserted-by":"crossref","first-page":"2049","DOI":"10.1212\/WNL.43.10.2049","article-title":"Type IIa (\u2018anti-Hu\u2019) antineuronal antibodies produce destruction of rat cerebellar granule neurons in vitro","volume":"43","author":"Greenlee","year":"1993","journal-title":"Neurology"},{"key":"10.1054\/mehy.1998.0771_RF39","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1007\/BF00309627","article-title":"Uptake of systemically administered human anticerebellar antibody by rat Purkinje cells following blood-brain barrier disruption","volume":"89","author":"Greenlee","year":"1995","journal-title":"Acta Neuropathol (Berl)"},{"key":"10.1054\/mehy.1998.0771_RF40","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1007\/s004010050460","article-title":"Inflammatory infiltrates and complete absence of Purkinje cells in anti-Yo-associated paraneoplastic cerebellar degeneration","volume":"91","author":"Verschuuren","year":"1996","journal-title":"Acta Neuropathol (Berl)"},{"key":"10.1054\/mehy.1998.0771_RF41","doi-asserted-by":"crossref","first-page":"1406","DOI":"10.1523\/JNEUROSCI.17-04-01406.1997","article-title":"A post-transcriptional regulatory mechanism restricts expression of the paraneoplastic cerebellar degeneration antigen cdr2 to immune privileged tissues","volume":"17","author":"Corradi","year":"1997","journal-title":"J Neurosci"},{"key":"10.1054\/mehy.1998.0771_RF42","doi-asserted-by":"crossref","first-page":"468","DOI":"10.1111\/j.1365-2990.1997.tb01323.x","article-title":"Sub-acute cerebellar degeneration with anti-Yo autoantibodies: immunohistochemical analysis of the immune reaction in the central nervous system","volume":"23","author":"Giometto","year":"1997","journal-title":"Neuropathol Appl Neurobiol"},{"key":"10.1054\/mehy.1998.0771_RF43","doi-asserted-by":"crossref","first-page":"2153","DOI":"10.1002\/(SICI)1097-0142(19961115)78:10<2153::AID-CNCR16>3.0.CO;2-Y","article-title":"Autoimmune paraneoplastic cerebellar degeneration in ovarian carcinoma patients treated with plasmapheresis and immunoglobulin. A case report","volume":"78","author":"David","year":"1996","journal-title":"Cancer"},{"key":"10.1054\/mehy.1998.0771_RF44","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1016\/0303-8467(95)00005-5","article-title":"Trial to establish an animal model of paraneoplastic cerebellar degeneration with anti-Yo antibody. 1. Mouse strains bearing different MHC molecules produce antibodies on immunization with recombinant Yo protein, but do not cause Purkinje cell loss","volume":"97","author":"Tanaka","year":"1995","journal-title":"Clin Neuro Neurosurg"},{"key":"10.1054\/mehy.1998.0771_RF45","first-page":"271","article-title":"Immunodeficiency in ataxia-telangiectasia","volume":"11","author":"Biggar","year":"1975","journal-title":"Birth Defects"},{"key":"10.1054\/mehy.1998.0771_RF46","first-page":"233","article-title":"Sequence of cellular events in cerebellar ontogeny relevant to expression of neuronal abnormalities in ataxia-telangiectasia","volume":"19","author":"Vinters","year":"1985","journal-title":"Kroc Foundation Series"},{"key":"10.1054\/mehy.1998.0771_RF47","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1038\/314053a0","article-title":"Demonstration by monoclonal antibodies that carbohydrate structures of glycoproteins and glycolipids are onco-developmental antigens","volume":"314","author":"Feizi","year":"1985","journal-title":"Nature"},{"key":"10.1054\/mehy.1998.0771_RF48","doi-asserted-by":"crossref","first-page":"3148","DOI":"10.1523\/JNEUROSCI.17-09-03148.1997","article-title":"Maternally derived immunoglobulin light chain is present in the fetal mammalian CNS","volume":"17","author":"Weiner","year":"1997","journal-title":"J Neurosci"},{"key":"10.1054\/mehy.1998.0771_RF49","first-page":"177","article-title":"Developmental mechanisms of neural network in the cerebellar system","volume":"15","author":"Yuasa","year":"1995","journal-title":"Nihon Shinkei Seishin Yakurigaku Zasshi"},{"key":"10.1054\/mehy.1998.0771_RF50","doi-asserted-by":"crossref","first-page":"814","DOI":"10.1001\/archneur.1995.00540320098016","article-title":"Immunological and clinical response to immunosuppressive treatment in paraneoplastic cerebellar degeneration","volume":"52","author":"Stark","year":"1995","journal-title":"Arch Neurol"}],"container-title":["Medical Hypotheses"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S030698779890771X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S030698779890771X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,5]],"date-time":"2025-10-05T19:11:07Z","timestamp":1759691467000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S030698779890771X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1999,5]]},"references-count":50,"journal-issue":{"issue":"5","published-print":{"date-parts":[[1999,5]]}},"alternative-id":["S030698779890771X"],"URL":"https:\/\/doi.org\/10.1054\/mehy.1998.0771","relation":{},"ISSN":["0306-9877"],"issn-type":[{"type":"print","value":"0306-9877"}],"subject":[],"published":{"date-parts":[[1999,5]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Neurodegeneration in ataxia\u2013telangiectasia is caused by horror autotoxicus","name":"articletitle","label":"Article Title"},{"value":"Medical Hypotheses","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1054\/mehy.1998.0771","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"converted-article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 1999 Harcourt Publishers Ltd. All rights reserved.","name":"copyright","label":"Copyright"}]}}