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題 名 | Nebulin在骨骼肌的分子結構及生理病理學=Nebulin: Molecular Structure and Pathophysiology in Skeletal Muscle |
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作 者 | 陳思萍; 林自勇; 陳文詮; 鄭麗菁; 陳頤之; | 書刊名 | 長庚科技學刊 |
卷 期 | 4 民94.12 |
頁 次 | 頁113-130 |
分類號 | 398.1 |
關鍵詞 | 肌動蛋白; 肌原纖維蛋白; 骨骼肌; 細肌絲; Actin; Myofibrillar protein; Nebulin; Skeletal muscle; Thin filament; |
語 文 | 中文(Chinese) |
中文摘要 | Nebulin (舊稱band3蛋白質) ,為一巨大的肌原纖維蛋白質,分子量約為600-900kDa,佔骨骼肌肌原纖維蛋白質總量3-5%。單一Nebulin分子為細長狀且無擴展性的結構,長度約為1.0-1.25微米,Nebulin被稱為骨骼肌的第四肌絲。Nebulin纏繞整個細肌絲的全長,其C端崁入Z線,其N端延伸至細肌絲靠近M線之末端處。成人的nebulin蛋白質結構分成四個區域:(1)一個獨特的N端區域;(2)185個複本(內含有35個氨基酸而形成的bulin組件) ,包含M1至M8以及M163至M185為單一重複構造,M9至M162為七個組件的超重複構造;(3)一個富含絲氯酸的鍊環構造;(4)位於C端的一個SH3區域。本文將介紹nebulin的發現過程,以及綜合許多文獻以記述nebulin為細肌絲的規尺。並且,更進一步分析近年來的實驗數據,以了解nebulin是一種具有多種功能的肌原纖維蛋白質,其在訊息傳遞和調節肌肉的收縮作用中扮演著重要的角色,同時也探討因nebulin基因突變而引起的細棒狀肌病,以及在棄用型萎縮症、激烈運動造成的肌肉損傷與酒精性肌肉病變等疾病中,顯示nebulin分子的蛋白質含量有顯著地降低的現象。 |
英文摘要 | Nebulin (formerly called band 3 protein), giant myofibrillar protein (600-900 kDa), comprised 3-5% of the myofibrillar protein of skeletal musc1e. A single nebulin molecule has long (about 1.0-1.25μm) and inextensible structure, and forms the fourth filament system of skeletal myofibrils. Nebulin spans the full length of thin filaments with its C-terminus anchored at the Z-line and its N-terminus extends to the thin filament pointed ends at the M-line. Adult human nebulin consists of four domains: (1) a unique N-terminal domain; (2) 185 copies of 35 residue nebulin modules (M1-185) that are either single repeats (M1-M8, M163-185) or seven-module super repeats (M9-162); (3) a serine-rich linker; (4) a SH3 domain at the C terminus. This review artic1e describes the discovery of nebulin and summarizes the several studies implicating nebulin as a thin filament ruler. Furthermore, novel data revealed that nebulin is a multifunctional myofibrillar protein, playing roles in signal transduction and muscle contractile regulation. We also discuss the mutation in nebulin gene associated with autosomal recessive nemaline myopathy, and a significant decline in protein levels of nebulin molecules existed in disuse atrophy, intense exercise-induced muscle damage and alcoholic myopathy. |
本系統中英文摘要資訊取自各篇刊載內容。