Cystine accumulation in the CNS results in severe age-related memory decits.pdf

Cystine accumulation in the CNS results in severe age-related memory decits.pdf

  1. 1、本文档共14页,可阅读全部内容。
  2. 2、原创力文档(book118)网站文档一经付费(服务费),不意味着购买了该文档的版权,仅供个人/单位学习、研究之用,不得用于商业用途,未经授权,严禁复制、发行、汇编、翻译或者网络传播等,侵权必究。
  3. 3、本站所有内容均由合作方或网友上传,本站不对文档的完整性、权威性及其观点立场正确性做任何保证或承诺!文档内容仅供研究参考,付费前请自行鉴别。如您付费,意味着您自己接受本站规则且自行承担风险,本站不退款、不进行额外附加服务;查看《如何避免下载的几个坑》。如果您已付费下载过本站文档,您可以点击 这里二次下载
  4. 4、如文档侵犯商业秘密、侵犯著作权、侵犯人身权等,请点击“版权申诉”(推荐),也可以打举报电话:400-050-0827(电话支持时间:9:00-18:30)。
查看更多
Cystine accumulation in the CNS results in severe age-related memory decits

NA t m t r c O C t ? K 1 g d a t D f M f 0 dARTICLE IN PRESSBA-6928; No. of Pages 14 Neurobiology of Aging xxx (2007) xxx–xxx Cystine accumulation in the CNS results in severe age-related memory deficits Tangui Maurice a,b,c, Claire Hippert d,e, Nicolas Serratrice d,e, Gre?gor Dubois d,e, Chantal Jacquet d,e, Corinne Antignac f,g, Eric J. Kremer d,e, Vasiliki Kalatzis d,e,? a Inserm U710, 34095 Montpellier, France b EPHE, 75017 Paris, France c Universite? de Montpellier II, 34095 Montpellier, France d Institut de Ge?ne?tique Mole?culaire de Montpellier, CNRS, 34293 Montpellier, France e Universite?s Montpellier 1 II, 34293 Montpellier, France f Inserm U574, 75015 Paris, France g Universite? Paris Descartes, Faculte? de Me?decine Rene? Descartes, UMRS574, 75015 Paris, France Received 11 April 2007; received in revised form 21 August 2007; accepted 18 September 2007 bstract Cystinosis is a lysosomal storage disorder characterised by progressive cystine accumulation. The causative gene,CTNS, encodes cystinosin, he lysosomal cystine transporter. Neurological deterioration is one of the last symptoms to appear and the least well characterised. Visuospatial emory deficits have been documented in patients. To determine whether the cystinosis mouse model presents similar anomalies, we studied he learning and memory abilities of young and middle-agedCtns?/? mice. We did not detect deficits in youngCtns?/? mice. In contrast, spatial eference and working memory deficits were detected in middle-aged Ctns?/? mice. Elevated cystine levels were detected in the hippocampus, erebellum, forebrain and brainstem of all Ctns?/? mice, which increased with age and were consistent with the appearance of impairments. ur results strongly suggest that the cystinosis-associated CNS anomalies are due to progressive cystine accumulation. Furthermore, the tns?/? mice serve as a model to investigate the evolution of these anomalies and test the efficiency of existing and novel treatments to cros

文档评论(0)

l215322 + 关注
实名认证
内容提供者

该用户很懒,什么也没介绍

1亿VIP精品文档

相关文档