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HACD1 B0YJ81

极长链(3R)-3-羟脂酰-CoA脱水酶1 (EC 4.2.1.134) (3-羟脂酰-CoA脱水酶1) (HACD1) (牙骨质附着蛋白) (CAP) (蛋白酪氨酸磷酸酶样成员A)

蛋白质信息 (UniProt)

极长链(3R)-3-羟脂酰-CoA脱水酶1 (EC 4.2.1.134) (3-羟脂酰-CoA脱水酶1) (HACD1) (牙骨质附着蛋白) (CAP) (蛋白酪氨酸磷酸酶样成员A)

B0YJ81

功能描述

[Isoform 1]: 催化长链脂肪酸延伸循环四个反应中的第三个反应。这一内质网结合的酶促过程,使得在每个循环中向长链和极长链脂肪酸/VLCFAs的碳链添加两个碳原子。该酶在脂肪酸延伸的每个循环内,催化3-羟基脂酰-CoA中间体脱水生成反式-2,3-烯脂酰-CoA。因此,它参与生成不同链长的VLCFAs,这些VLCFAs作为膜脂和脂质介质的前体参与多种生物过程。 {ECO:0000269|PubMed:18554506}.; FUNCTION: [Isoform 2]: 在牙齿发育中,可能在募集和分化有助于牙骨质形成的细胞方面发挥作用。也可能结合羟基磷灰石并调节其晶体成核以形成牙骨质。 {ECO:0000269|PubMed:22067203}.

组织特异性

Isoform 1 is highly expressed in the myocardium, and to a lesser extent in skeletal and smooth muscular tissues including those from stomach, jejunum, and bladder. Also detected in gingival fibroblasts, periodontal ligament cells, osteoblasts and cementoblasts (PubMed:11054553, PubMed:22067203). Isoform 2 is specifically expressed by cementoblasts but also detected in periodontal ligament cells, heart, liver and kidney (at protein level) (PubMed:22067203).

亚细胞定位

[Isoform 1]: Endoplasmic reticulum membrane

关键词

Alternative splicing Developmental protein Disease variant Endoplasmic reticulum Fatty acid biosynthesis Fatty acid metabolism Glycoprotein Lipid biosynthesis Lipid metabolism Lyase