{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T21:01:49Z","timestamp":1790370109193,"version":"4.1.0"},"reference-count":33,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2001,4,30]],"date-time":"2001-04-30T00:00:00Z","timestamp":988588800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Neuroscience Research"],"published-print":{"date-parts":[[2001,5,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Laboratory mice carrying the nonfunctional xeroderma pigmentosum group G gene (the mouse counterpart of the human <jats:italic>XPG<\/jats:italic> gene) alleles have been generated by using gene\u2010targeting and embryonic stem cell technology. Homozygote animals of this autosomal recessive disease exhibited signs and symptoms, such as postnatal growth retardation, reduced levels of activity, progressive ataxia and premature death, similar to the clinical manifestations of Cockayne syndrome (CS). Histological analysis of the cerebellum revealed multiple pyknotic cells in the Purkinje cell layer of the <jats:italic>xpg<\/jats:italic> homozygotes, which had atrophic cell bodies and shrunken nuclei. Further examination by an immunohistochemistry for calbindin\u2010D 28k (CaBP) showed that a large number of immunoreactive Purkinje cells were atrophic and their dendritic trees were smaller and shorter than in wild\u2010type littermates. These results indicated a marked degeneration of Purkinje cells in the <jats:italic>xpg<\/jats:italic> mutant cerebellum. Study by in situ detection of DNA fragmentation in the cerebellar cortex demonstrated that some deoxynucleotidyl transferase (TdT)\u2010mediated dUTP\u2010biotin in situ nick labeling (TUNEL)\u2010positive cells appeared in the granule layer of the mutant mice, but few cell deaths were confirmed in the Purkinje layer. These results suggested Purkinje cell degeneration in the mutant cerebellum was underway, in which much Purkinje cell death had not appeared, and the appearance of some abnormal cerebellar symptoms in the <jats:italic>xpg<\/jats:italic>\u2010deficient mice was not only due to a marked Purkinje cell degeneration, but also to damage of other cells. J. Neurosci. Res. 64:348\u2013354, 2001. \u00a9 2001 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jnr.1085","type":"journal-article","created":{"date-parts":[[2002,8,25]],"date-time":"2002-08-25T20:13:04Z","timestamp":1030306384000},"page":"348-354","source":"Crossref","is-referenced-by-count":27,"title":["Purkinje cell degeneration in mice lacking the xeroderma pigmentosum group G 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