{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T12:02:52Z","timestamp":1789646572932,"version":"4.0.0"},"reference-count":22,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2011,3,29]],"date-time":"2011-03-29T00:00:00Z","timestamp":1301356800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2012,2]]},"abstract":"<jats:p>\n                    Ataxia\u2010telangiectasia (A\u2010T) is a heritable disorder of cerebellar ataxia and oculocutaneous telangiectasias caused by mutation of the\n                    <jats:italic>ATM<\/jats:italic>\n                    gene. The most prominent and consistent neuropathologic finding in the disorder is cerebellar cortical degeneration involving significant loss of granule and Purkinje cells. Several past autopsy studies of A\u2010T patients have also noted large\u2010bodied cells located within the molecular layer of the cerebellar cortex and, noting similarities in morphology between these cells and Purkinje cells, hypothesized that the cells were heterotopic Purkinje cells. This study aimed to test this hypothesis using an antibody that labels Purkinje cells, and also to investigate other cell types in the degenerating cerebellar cortex in A\u2010T. Using the anti\u2010calbindin D\u201028K antibody to label Purkinje cells in cerebellar tissue from five A\u2010T patients and five age\u2010 and sex\u2010matched controls, the study found calbindin\u2010positive heterotopic Purkinje cells in the molecular layer occurring at a significantly higher rate in A\u2010T patients than in controls (\n                    <jats:italic>P<\/jats:italic>\n                    \u2003=\u20030.012). Further immunohistochemistry with the anti\u2010Iba\u20101 and anti\u2010parvalbumin antibodies showed, respectively, an increase in microglial activity (\n                    <jats:italic>P<\/jats:italic>\n                    \u2003=\u20030.14) and stellate\u2010cell density (\n                    <jats:italic>P<\/jats:italic>\n                    \u2003=\u20030.0048) in the cerebellar cortex of A\u2010T patients versus controls. These data add to the as yet unresolved debate over the origin and significance of heterotopic Purkinje cells in A\u2010T.\n                  <\/jats:p>","DOI":"10.1111\/j.1440-1789.2011.01210.x","type":"journal-article","created":{"date-parts":[[2011,3,29]],"date-time":"2011-03-29T05:29:21Z","timestamp":1301376561000},"page":"23-29","source":"Crossref","is-referenced-by-count":24,"title":["Heterotopic Purkinje cells in ataxia\u2010telangiectasia"],"prefix":"10.1111","volume":"32","author":[{"given":"Alexander R.","family":"Bottini","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard A.","family":"Gatti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Wirenfeldt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harry V.","family":"Vinters","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2011,3,29]]},"reference":[{"key":"e_1_2_6_2_2","first-page":"1","article-title":"Ataxia\u2010telangiectasia: an overview","volume":"19","author":"Boder E","year":"1985","journal-title":"Kroc Found Ser"},{"key":"e_1_2_6_3_2","first-page":"541","article-title":"Contribution a l'independence de l'athetose double idiopathique et congenitale. Atteinte familiale, syndrome dystrophique, signe de reseau vasculaire conjonctival, integrite physique","volume":"1","author":"Syllaba K","year":"1926","journal-title":"Rev Neurol"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/nrm2514"},{"key":"e_1_2_6_5_2","first-page":"15","article-title":"Ataxia\u2010telangiectasia. A familial syndrome of progressive cerebellar ataxia, oculocutaneous telangiectasia and frequent pulmonary infection. A preliminary report on 7 children, an autopsy, and a case history","volume":"9","author":"Boder E","year":"1957","journal-title":"Univ S Calif Med Bull"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.immunol.15.1.177"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(64)91209-7"},{"key":"e_1_2_6_8_2","first-page":"8","article-title":"Ataxia\u2010telangiectasia. (Clinical and immunological aspects)","volume":"13","author":"Boder E","year":"1970","journal-title":"Psychiatr Neurol Med Psychol Beih"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197006182822503"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-199103000-00003"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.1967.02090270073006"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1038\/258427a0"},{"key":"e_1_2_6_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(72)92717-1"},{"key":"e_1_2_6_14_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.3975628"},{"key":"e_1_2_6_15_2","doi-asserted-by":"publisher","DOI":"10.1038\/336577a0"},{"key":"e_1_2_6_16_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.7792600"},{"key":"e_1_2_6_17_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.29.6.489"},{"key":"e_1_2_6_18_2","first-page":"233","article-title":"Sequence of cellular events in cerebellar ontogeny relevant to expression of neuronal abnormalities in ataxia\u2010telangiectasia","volume":"19","author":"Vinters HV","year":"1985","journal-title":"Kroc Found Ser"},{"key":"e_1_2_6_19_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.97.7.3336"},{"key":"e_1_2_6_20_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneurol.2007.8"},{"key":"e_1_2_6_21_2","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.22056"},{"key":"e_1_2_6_22_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2009.02.015"},{"key":"e_1_2_6_23_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410130312"}],"container-title":["Neuropathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1440-1789.2011.01210.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1440-1789.2011.01210.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,29]],"date-time":"2023-10-29T21:30:46Z","timestamp":1698615046000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1440-1789.2011.01210.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,3,29]]},"references-count":22,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2012,2]]}},"alternative-id":["10.1111\/j.1440-1789.2011.01210.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1440-1789.2011.01210.x","archive":["Portico"],"relation":{},"ISSN":["0919-6544","1440-1789"],"issn-type":[{"value":"0919-6544","type":"print"},{"value":"1440-1789","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,3,29]]}}}