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The structural basis of ZMPSTE24-dependent laminopathies

Abstract:

Mutations in the nuclear membrane zinc metalloprotease ZMPSTE24 lead to diseases of lamin processing (laminopathies), such as the premature aging disease progeria and metabolic disorders. ZMPSTE24 processes prelamin A, a component of the nuclear lamina intermediate filaments, by cleaving it at two sites. Failure of this processing results in accumulation of farnesylated, membrane-associated prelamin A. The 3.4 angstrom crystal structure of human ZMPSTE24 has a seven transmembrane α-helical ba...

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Publisher copy:
10.1126/science.1231513

Authors


Journal:
Science More from this journal
Volume:
340
Issue:
6127
Pages:
1604-1607
Publication date:
2013-03-29
DOI:
EISSN:
1095-9203
ISSN:
0036-8075
Language:
English
Pubs id:
pubs:395221
UUID:
uuid:064f9553-0aec-4ab9-a5ea-979ec4f82394
Local pid:
pubs:395221
Source identifiers:
395221
Deposit date:
2013-11-17

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