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核心蛋白聚糖的硫酸皮肤素链缺失不影响小鼠发育。

Absence of the dermatan sulfate chain of decorin does not affect mouse development.

作者信息

Moffatt Pierre, Geng Yeqing, Lamplugh Lisa, Nanci Antonio, Roughley Peter J

机构信息

Research Center, Shriners Hospitals for Children - Canada, 1003 Boulevard Décarie, Montréal, H4A 0A9, QC, Canada.

Department of Human Genetics, Faculty of Medicine, McGill University, Montreal, QC, Canada.

出版信息

J Negat Results Biomed. 2017 Apr 17;16(1):7. doi: 10.1186/s12952-017-0074-3.

DOI:10.1186/s12952-017-0074-3
PMID:28412940
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5393030/
Abstract

BACKGROUND

In vitro studies suggest that the multiple functions of decorin are related to both its core protein and its dermatan sulfate chain. To determine the contribution of the dermatan sulfate chain to the functional properties of decorin in vivo, a mutant mouse whose decorin lacked a dermatan sulfate chain was generated.

RESULTS

Homozygous mice expressing only the decorin core protein developed and grew in a similar manner to wild type mice. In both embryonic and postnatal mice, all connective tissues studied, including cartilage, skin and cornea, appeared to be normal upon histological examination, and their collagen fibrils were of normal diameter and organization. In addition, abdominal skin wounds healed in an identical manner in the mutant and wild type mice.

CONCLUSIONS

The absence of a dermatan sulfate chain on decorin does not appear to overtly influence its functional properties in vivo.

摘要

背景

体外研究表明,核心蛋白聚糖的多种功能与其核心蛋白及其硫酸皮肤素链均有关。为了确定硫酸皮肤素链对体内核心蛋白聚糖功能特性的作用,制备了一种核心蛋白聚糖缺乏硫酸皮肤素链的突变小鼠。

结果

仅表达核心蛋白聚糖核心蛋白的纯合小鼠的发育和生长方式与野生型小鼠相似。在胚胎期和出生后的小鼠中,所有研究的结缔组织,包括软骨、皮肤和角膜,经组织学检查似乎均正常,其胶原纤维直径和组织结构也正常。此外,突变小鼠和野生型小鼠腹部皮肤伤口的愈合方式相同。

结论

核心蛋白聚糖上缺乏硫酸皮肤素链似乎并未明显影响其在体内的功能特性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee62/5393030/c8934db84c79/12952_2017_74_Fig6_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee62/5393030/cdca064c3745/12952_2017_74_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee62/5393030/a502ee007181/12952_2017_74_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee62/5393030/82bd484312da/12952_2017_74_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee62/5393030/40a16e767a2a/12952_2017_74_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee62/5393030/51c4cddaf7cb/12952_2017_74_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee62/5393030/c8934db84c79/12952_2017_74_Fig6_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee62/5393030/cdca064c3745/12952_2017_74_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee62/5393030/a502ee007181/12952_2017_74_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee62/5393030/82bd484312da/12952_2017_74_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee62/5393030/40a16e767a2a/12952_2017_74_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee62/5393030/51c4cddaf7cb/12952_2017_74_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee62/5393030/c8934db84c79/12952_2017_74_Fig6_HTML.jpg

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Matrix Biol. 2016 Sep;55:7-21. doi: 10.1016/j.matbio.2016.09.009. Epub 2016 Sep 30.
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Analysis of protein-coding genetic variation in 60,706 humans.对60706名人类的蛋白质编码基因变异进行分析。
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Forced exercise-induced osteoarthritis is attenuated in mice lacking the small leucine-rich proteoglycan decorin.
糖胺聚糖和蛋白聚糖在眼表发育、稳态及病理过程中的分布与功能
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Impaired proteoglycan glycosylation, elevated TGF-β signaling, and abnormal osteoblast differentiation as the basis for bone fragility in a mouse model for gerodermia osteodysplastica.成骨发育不全性皮肤骨肥厚症小鼠模型中糖基化蛋白聚糖受损、TGF-β 信号转导升高和破骨细胞分化异常导致的骨脆弱。
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