Palmitoylation and depalmitoylation defects

Palmitoylation and depalmitoylation defects
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DOI:
10.1007/s10545-014-9753-0
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发表时间:
2015-01-01
影响因子:
4.2
通讯作者:
Hornemann, Thorsten
Hornemann, Thorsten
中科院分区:
医学2区
文献类型:
--
作者:
Hornemann, Thorsten

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棕榈酰化描述了16个碳原子的脂肪酸与靶蛋白的酶促连接。这种脂化事件发生在所有真核生物中,并且可以是可逆的(S-棕榈酰化)或不可逆的(N-棕榈酰化)性质。特别地,S-棕榈酰化由两种相反类型的酶动态调节,所述酶从蛋白质添加(棕榈酰酰基转移酶- PAT)或去除(酰基蛋白硫酯酶)棕榈酸酯。蛋白质棕榈酰化是一个重要的过程,动态调节许多神经元蛋白质在特定亚细胞位点的组装和区室化。调节蛋白质棕榈酰化的酶对于几个生物过程是至关重要的。迄今为止,已报道八个棕榈酰化相关基因与人类疾病相关。本文就棕榈酰化/脱棕榈酰化过程中的缺陷所引起的病理变化作一综述。
Palmitoylation describes the enzymatic attachment of a 16-carbon atom fatty acid to a target protein. Such lipidation events occur in all eukaryotes and can be of reversible (S-palmitoylation) or irreversible (N-palmitoylation) nature. In particular S-palmitoylation is dynamically regulated by two opposing types of enzymes which add (palmitoyl acyltransferases - PAT) or remove (acyl protein thioesterases) palmitate from proteins. Protein palmitoylation is an important process that dynamically regulates the assembly and compartmentalization of many neuronal proteins at specific subcellular sites. Enzymes that regulate protein palmitoylation are critical for several biological processes. To date, eight palmitoylation related genes have been reported to be associated with human disease. This review intends to give an overview on the pathological changes which are associated with defects in the palmitoylation/depalmitoylation process.