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人眼外肌中肌球蛋白重链和肌球蛋白结合蛋白C亚型的不协调表达。

Uncoordinated expression of myosin heavy chains and myosin-binding protein C isoforms in human extraocular muscles.

作者信息

Kjellgren Daniel, Stål Per, Larsson Lars, Fürst Dieter, Pedrosa-Domellöf Fatima

机构信息

Department of Clinical Sciences, Ophthalmology, Umeå University, Umeå, Sweden; the.

出版信息

Invest Ophthalmol Vis Sci. 2006 Oct;47(10):4188-93. doi: 10.1167/iovs.05-1496.

DOI:10.1167/iovs.05-1496
PMID:17003405
Abstract

PURPOSE

To examine the distribution of myosin-binding protein C (MyBP-C) in human extraocular muscles (EOMs) and to correlate the myosin heavy chain (MyHC) and the MyBP-C composition of the fibers.

METHODS

Samples from 17 EOMs, 3 levator palpebrae (LP), and 6 limb muscles were analyzed with SDS-PAGE and immunoblot or processed for immunocytochemistry with monoclonal antibodies (mAbs) against MyBP-C-fast, MyBP-C-slow, MyHCIIa, MyHCI, MyHCsto, MyHCalpha-cardiac, and MyHCemb.

RESULTS

In the limb muscle samples, fast fibers were labeled with anti-MyBP-C-fast and anti-MyBP-C-slow, whereas the slow fibers were immunostained with anti-MyBP-C-slow only, in accordance with previous studies. In 11 EOM samples MyBP-C-fast was not detected, and weak staining with anti-MyBP-C-fast was seen only in a few fibers in the proximal part of 2 muscles. The mAb against MyBP-C-slow labeled all fibers, but fibers containing MyHCI were generally more strongly stained. In the levator palpebrae, immunostaining with anti-MyBP-C-fast was present in some fibers labeled with anti-MyHCIIa and/or anti-MyHCeom. MyBP-C-fast and -intermediate were not detected biochemically in the EOMs.

CONCLUSIONS

The lack of MyBP-C-fast and intermediate is an additional feature of the human EOM allotype. The true EOMs have a unique myofibrillar protein isoform composition reflecting their special structural and functional properties. The levator palpebrae muscle phenotype is intermediate between that of the EOMs and the limb muscles.

摘要

目的

研究肌球蛋白结合蛋白C(MyBP-C)在人眼外肌(EOMs)中的分布,并将纤维的肌球蛋白重链(MyHC)与MyBP-C组成进行关联。

方法

采用十二烷基硫酸钠-聚丙烯酰胺凝胶电泳(SDS-PAGE)和免疫印迹法分析17条眼外肌、3条提上睑肌(LP)和6条肢体肌肉的样本,或用抗MyBP-C-fast、MyBP-C-slow、MyHCIIa、MyHCI、MyHCsto、MyHCalpha-心脏型和MyHCemb的单克隆抗体(mAbs)进行免疫细胞化学处理。

结果

在肢体肌肉样本中,快肌纤维用抗MyBP-C-fast和抗MyBP-C-slow标记,而慢肌纤维仅用抗MyBP-C-slow免疫染色,与先前研究一致。在11个眼外肌样本中未检测到MyBP-C-fast,仅在2条肌肉近端的少数纤维中观察到抗MyBP-C-fast的弱染色。抗MyBP-C-slow的单克隆抗体标记了所有纤维,但含有MyHCI的纤维通常染色更强。在提上睑肌中,抗MyBP-C-fast的免疫染色存在于一些用抗MyHCIIa和/或抗MyHCeom标记的纤维中。在眼外肌中未通过生化方法检测到MyBP-C-fast和中间型。

结论

缺乏MyBP-C-fast和中间型是人类眼外肌异型的另一个特征。真正的眼外肌具有独特的肌原纤维蛋白异构体组成,反映了它们特殊的结构和功能特性。提上睑肌的表型介于眼外肌和肢体肌肉之间。

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