{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T15:08:20Z","timestamp":1770822500491,"version":"3.50.1"},"reference-count":0,"publisher":"Rockefeller University Press","issue":"6","content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1991,9,15]]},"abstract":"<jats:p>Mice homozygous for the nb mutation (Chromosome 8) have a severe hemolytic anemia and develop a psychomotor disorder at 6 mo of age. The nb\/nb mice are deficient in erythroid ankyrin (Ank-1) but, until the present study, the role of Ank-1 and of Ank-2 (brain ankyrin) in disease genesis was unknown. In normal erythroid tissues, we show that two major transcripts are expressed from Ank-1, and one of these is also present at high levels in the cerebellum. By in situ hybridization and immunocytochemistry, Ank-1 localizes to the cerebellar Purkinje cells and, to a lesser extent, the granule cells. In nb\/nb mice, Ank-1 transcripts are markedly reduced in both erythroid and neural tissue, and nb\/nb Purkinje cells and granule cells are nearly devoid of Ank-1. The neurological syndrome appears concurrently with a dramatic loss of Purkinje cells. Ank-2 maps to Chromosome 3 and its expression is unaffected by the nb mutation. We conclude that Ank-1 is specifically required for Purkinje cell stability and, in its absence, Purkinje cell loss and neurological symptoms appear.<\/jats:p>","DOI":"10.1083\/jcb.114.6.1233","type":"journal-article","created":{"date-parts":[[2004,5,15]],"date-time":"2004-05-15T00:18:20Z","timestamp":1084580300000},"page":"1233-1241","update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":79,"title":["Purkinje cell degeneration associated with erythroid ankyrin deficiency in nb\/nb mice."],"prefix":"10.1083","volume":"114","author":[{"given":"L L","family":"Peters","sequence":"first","affiliation":[{"name":"Jackson Laboratory, Bar Harbor, Maine 04609."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C S","family":"Birkenmeier","sequence":"additional","affiliation":[{"name":"Jackson Laboratory, Bar Harbor, Maine 04609."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R T","family":"Bronson","sequence":"additional","affiliation":[{"name":"Jackson Laboratory, Bar Harbor, Maine 04609."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R A","family":"White","sequence":"additional","affiliation":[{"name":"Jackson Laboratory, Bar Harbor, Maine 04609."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S E","family":"Lux","sequence":"additional","affiliation":[{"name":"Jackson Laboratory, Bar Harbor, Maine 04609."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E","family":"Otto","sequence":"additional","affiliation":[{"name":"Jackson Laboratory, Bar Harbor, Maine 04609."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"V","family":"Bennett","sequence":"additional","affiliation":[{"name":"Jackson Laboratory, Bar Harbor, Maine 04609."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A","family":"Higgins","sequence":"additional","affiliation":[{"name":"Jackson Laboratory, Bar Harbor, Maine 04609."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J E","family":"Barker","sequence":"additional","affiliation":[{"name":"Jackson Laboratory, Bar Harbor, Maine 04609."}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"291","published-online":{"date-parts":[[1991,9,15]]},"container-title":["The Journal of cell biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/rupress.org\/jcb\/article-pdf\/114\/6\/1233\/1465534\/1233.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/rupress.org\/jcb\/article-pdf\/114\/6\/1233\/1465534\/1233.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,7,22]],"date-time":"2023-07-22T01:34:21Z","timestamp":1689989661000},"score":1,"resource":{"primary":{"URL":"https:\/\/rupress.org\/jcb\/article\/114\/6\/1233\/14227\/Purkinje-cell-degeneration-associated-with"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1991,9,15]]},"references-count":0,"journal-issue":{"issue":"6","published-print":{"date-parts":[[1991,9,15]]}},"URL":"https:\/\/doi.org\/10.1083\/jcb.114.6.1233","relation":{},"ISSN":["0021-9525","1540-8140"],"issn-type":[{"value":"0021-9525","type":"print"},{"value":"1540-8140","type":"electronic"}],"subject":[],"published":{"date-parts":[[1991,9,15]]}}}