Abstract：
:Accumulation of misfolded proteins and alterations in the ubiquitin-proteasome pathway are associated with various neurodegenerative conditions of the CNS and PNS. Aggregates containing ubiquitin and peripheral myelin protein 22 (PMP22) have been observed in the Trembler J mouse model of Charcot-Marie-Tooth disease type 1A demyelinating neuropathy. In these nerves, the turnover rate of the newly synthesized PMP22 is reduced, suggesting proteasome impairment. Here we show evidence of proteasome impairment in Trembler J neuropathy samples compared with wild-type, as measured by reduced degradation of substrate reporters. Proteasome impairment correlates with increased levels of polyubiquitinated proteins, including PMP22, and the recruitment of E1, 20S and 11S to aggresomes formed either spontaneously due to the Trembler J mutation or upon proteasome inhibition. Furthermore, myelin basic protein, an endogenous Schwann cell proteasome substrate, associates with PMP22 aggregates in affected nerves. Together, our data show that in neuropathy nerves, reduced proteasome activity is coupled with the accumulation of ubiquitinated substrates, and the recruitment of proteasomal pathway constituents to aggregates. These results provide novel insights into the mechanism by which altered degradation of Schwann cell proteins may contribute to the pathogenesis of certain PMP22 neuropathies.
journal_name
J Neurochemjournal_title
Journal of neurochemistryauthors
Fortun J,Li J,Go J,Fenstermaker A,Fletcher BS,Notterpek Ldoi
10.1111/j.1471-4159.2004.02987.xkeywords:
subject
Has Abstractpub_date
2005-03-01 00:00:00pages
1531-41issue
6eissn
0022-3042issn
1471-4159pii
JNC2987journal_volume
92pub_type
杂志文章abstract：:Mutations in the presenilin 1 (PS1) gene are associated with autosomal dominant, early-onset, familial Alzheimer's disease and result in increased release of the hyperaggregatable 42-amino acid form of the amyloid beta-peptide (A(beta)42). To determine which subcellular compartments are potential source(s) of released...
journal_title：Journal of neurochemistry
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pub_type： 杂志文章
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
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更新日期：1986-01-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1046/j.1471-4159.1995.64041721.x
更新日期：1995-04-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
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更新日期：2005-08-01 00:00:00
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journal_title：Journal of neurochemistry
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doi：10.1046/j.1471-4159.1996.66041391.x
更新日期：1996-04-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.1983.tb00866.x
更新日期：1983-12-01 00:00:00
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pub_type： 杂志文章
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更新日期：2012-12-01 00:00:00
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pub_type： 杂志文章
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更新日期：1983-04-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1046/j.1471-4159.1996.66010169.x
更新日期：1996-01-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1111/j.1471-4159.1989.tb01873.x
更新日期：1989-04-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
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更新日期：1995-05-01 00:00:00
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journal_title：Journal of neurochemistry
pub_type： 杂志文章
doi：10.1046/j.1471-4159.2003.01592.x
更新日期：2003-03-01 00:00:00
