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缺乏乙酰肝素 N-脱乙酰酶/N-磺基转移酶 1 的小鼠。

Mice deficient in heparan sulfate N-deacetylase/N-sulfotransferase 1.

机构信息

Department of Medical Biochemistry and Microbiology, The Biomedical Center, Uppsala University, Uppsala, Sweden.

出版信息

Prog Mol Biol Transl Sci. 2010;93:35-58. doi: 10.1016/S1877-1173(10)93003-2.

DOI:10.1016/S1877-1173(10)93003-2
PMID:20807640
Abstract

Ndsts (N-deacetylase/N-sulfotransferases) are enzymes responsible for N-sulfation during heparan sulfate (HS) and heparin biosynthesis. In this review, basic features of the Ndst1 enzyme are covered and a brief description of HS biosynthesis and its regulation is presented. Effects of Ndst1 deficiency on embryonic development are described. These include immature lungs, craniofacial dysplasia and eye developmental defects, branching defect during lacrimal gland development, delayed mineralization of the skeleton, and reduced pericyte recruitment during vascular development. A brief account of the effects of Ndst1 deficiency in selective cell types in adult mice is also given.

摘要

Ndsts(N-脱乙酰基/N-磺基转移酶)是负责硫酸乙酰肝素(HS)和肝素生物合成过程中 N-磺化的酶。本综述涵盖了 Ndst1 酶的基本特征,并简要描述了 HS 的生物合成及其调控。还描述了 Ndst1 缺乏对胚胎发育的影响。这些影响包括肺不成熟、颅面发育不良和眼睛发育缺陷、泪腺发育过程中的分支缺陷、骨骼矿化延迟以及血管发育过程中周细胞募集减少。本文还简要介绍了 Ndst1 缺乏在成年小鼠某些特定细胞类型中的作用。

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