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nebulette
The ''NEBL'' gene encodes the protein Nebulette, a cardiac-specific isoform belonging to the nebulin family of proteins. This family is composed of 5 members: nebulette, nebulin, N-RAP, LASP-1 and LASP-2. Nebulette localizes to Z-discs of cardiac muscle and appears to regulate the length of actin thin filaments.
== Structure ==
Nebulette is a 116.4 kDa protein composed of 1014 amino acids.〔(【引用サイトリンク】 work = Cardiac Organellar Protein Atlas Knowledgebase (COPaKB) )〕 As a member of the nebulin family of proteins, nebulette is characterized by 35 amino acid stretches of ‘‘nebulin repeats’’, which are actin binding domains containing a conserved SDxxYK motif. Like nebulin, nebulette has an acidic region with unknown structure at its N-terminus, and a serine-rich region adjacent to an SH3 domain at its C-terminus. Though nebulette shares structural features with nebulin, nebulin is expressed preferentially in skeletal muscle and has an enormous size (600-900 kDa), while nebulette is expressed in cardiac muscle at Z-disc regions and is significantly smaller (roughly 1/6 of the size). Nebulette interacts with actin, tropomyosin, alpha-actinin. Xin, and XIRP2.
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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