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Crystal structures of wild-type and mutated cyclophilin B that causes hyperelastosis cutis in the American quarter horse.

作者信息

Boudko Sergei P, Ishikawa Yoshihiro, Lerch Thomas F, Nix Jay, Chapman Michael S, Bächinger Hans Peter

机构信息

Research Department, Shriners Hospital for Children, Portland, OR 97239, USA.

出版信息

BMC Res Notes. 2012 Nov 8;5:626. doi: 10.1186/1756-0500-5-626.

DOI:10.1186/1756-0500-5-626
PMID:23137129
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3522003/
Abstract

BACKGROUND

Hyperelastosis cutis is an inherited autosomal recessive connective tissue disorder. Affected horses are characterized by hyperextensible skin, scarring, and severe lesions along the back. The disorder is caused by a mutation in cyclophilin B.

RESULTS

The crystal structures of both wild-type and mutated (Gly6->Arg) horse cyclophilin B are presented. The mutation neither affects the overall fold of the enzyme nor impairs the catalytic site structure. Instead, it locally rearranges the flexible N-terminal end of the polypeptide chain and also makes it more rigid.

CONCLUSIONS

Interactions of the mutated cyclophilin B with a set of endoplasmic reticulum-resident proteins must be affected.

摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a9cd/3522003/247ffbd36329/1756-0500-5-626-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a9cd/3522003/247ffbd36329/1756-0500-5-626-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a9cd/3522003/247ffbd36329/1756-0500-5-626-1.jpg

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