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The ubiquitin-selective chaperone CDC-48/p97 links myosin assembly to human myopathy.

Abstract：

:Protein degradation in eukaryotes often requires the ubiquitin-selective chaperone p97 for substrate recruitment and ubiquitin-chain assembly. However, the physiological relevance of p97, and its role in developmental processes, remain unclear. Here, we discover an unanticipated function for CDC-48/p97 in myosin assembly and myofibril organization, both in Caenorhabditis elegans and humans. The developmentally regulated assembly of a CDC-48-UFD-2-CHN-1 complex links turnover of the myosin-directed chaperone UNC-45 to functional muscle formation. Our data suggest a similarly conserved pathway regulating myosin assembly in humans. Remarkably, mutations in human p97, known to cause hereditary inclusion-body myopathy, abrogate UNC-45 degradation and result in severely disorganized myofibrils, detrimental towards sarcomeric function. These results identify a key role for CDC-48/p97 in the process of myofibre differentiation and maintenance, which is abolished during pathological conditions leading to protein aggregation and inclusion-body formation in human skeletal muscle.

journal_name

Nat Cell Biol

journal_title

Nature cell biology

authors

Janiesch PC,Kim J,Mouysset J,Barikbin R,Lochmüller H,Cassata G,Krause S,Hoppe T

doi

10.1038/ncb1554

subject

Has Abstract

pub_date

2007-04-01 00:00:00

pages

379-90

issue

4

eissn

1465-7392

issn

1476-4679

pii

ncb1554

journal_volume

9

pub_type

杂志文章

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