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Science
Article . 2013 . Peer-reviewed
Data sources: Crossref
Science
Article . 2013
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The Structural Basis of ZMPSTE24-Dependent Laminopathies

Authors: Quigley, A; Dong, Y; Pike, A; Dong, L; Shrestha, L; Berridge, G; Stansfeld, P; +7 Authors

The Structural Basis of ZMPSTE24-Dependent Laminopathies

Abstract

Lamin Loppers The nuclear lamina provides mechanical stability to the nuclear envelope and is involved in regulation of cellular processes such as DNA replication. Defects in the nuclear lamina lead to diseases such as progeria and metabolic disorders. One of the components of the nuclear lamina, lamin A, undergoes a complex maturation process. A key player is an inner nuclear membrane zinc metalloprotease (ZMP) that is responsible for two proteolysis steps (see the Perspective by Michaelis and Hrycyna ). Quigley et al. (p. 1604 ) report the crystal structure of human ZMPSTE24 and Pryor et al. (p. 1600 ) that of the yeast homolog Ste24p. The structures provide insight into the mechanism of catalysis and into why mutations in ZMPSTE24 lead to laminopathies.

Country
United Kingdom
Keywords

Protein Conformation, Molecular Sequence Data, Thermolysin, Membrane Proteins, Metalloendopeptidases, Nuclear Proteins, Crystallography, X-Ray, Lamin Type A, Substrate Specificity, Progeria, Catalytic Domain, Humans, Amino Acid Sequence, Protein Precursors, Metabolism, Inborn Errors

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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
99
Top 10%
Top 10%
Top 1%
Green