The structural basis of ZMPSTE24-dependent laminopathies.

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 s...

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Main Authors: Quigley, A, Dong, Y, Pike, A, Dong, L, Shrestha, L, Berridge, G, Stansfeld, P, Sansom, MS, Edwards, A, Bountra, C, von Delft, F, Bullock, A, Burgess-Brown, N, Carpenter, E
格式: Journal article
语言:English
出版: 2013