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首页> 美国卫生研究院文献>The Journal of Biological Chemistry >Palmitoylation of Progressive Rod-Cone Degeneration (PRCD) Regulates Protein Stability and Localization
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Palmitoylation of Progressive Rod-Cone Degeneration (PRCD) Regulates Protein Stability and Localization

机译：渐进性杆状锥变性（PRCD）的棕榈酰化调节蛋白质的稳定性和定位

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摘要

Progressive rod-cone degeneration (PRCD) is a photoreceptor outer segment (OS) disc-specific protein with unknown function that is associated with retinitis pigmentosa (RP). The most common mutation in PRCD linked with severe RP phenotype is substitution of the only cysteine to tyrosine (C2Y). In this study, we find that PRCD is post-translationally modified by a palmitoyl lipid group at the cysteine residue linked with RP. Disrupting PRCD palmitoylation either chemically or by genetically eliminating the modified cysteine dramatically affects the stability of PRCD. Furthermore, in vivo electroporation of PRCD C2Y mutant in the mouse retina demonstrates that the palmitoylation of PRCD is important for its proper localization in the photoreceptor OS. Mutant PRCD C2Y was found in the inner segment in contrast to normal localization of WT PRCD in the OS. Our results also suggest that zDHHC3, a palmitoyl acyltransferase (PAT), catalyzes the palmitoylation of PRCD in the Golgi compartment. In conclusion, we find that the palmitoylation of PRCD is crucial for its trafficking to the photoreceptor OS and mislocalization of this protein likely leads to RP-related phenotypes.
机译：进行性视锥变性（PRCD）是一种功能未知的感光细胞外节（OS）盘特异性蛋白，与色素性视网膜炎（RP）相关。与严重的RP表型有关的PRCD中最常见的突变是将唯一的半胱氨酸替换为酪氨酸（C2Y）。在这项研究中，我们发现PRCD在与RP连接的半胱氨酸残基处被棕榈酰脂质基团翻译后修饰。通过化学方式或通过基因消除修饰的半胱氨酸破坏PRCD的棕榈酰化作用极大地影响了PRCD的稳定性。此外，在小鼠视网膜中PRCD C2Y突变体的体内电穿孔表明，PRCD的棕榈酰化对其在感光体OS中的正确定位很重要。与WT PRCD在操作系统中的正常定位相反，在内部片段中发现了突变的PRCD C2Y。我们的结果还表明，zDHHC3是一种棕榈酰酰基转移酶（PAT），可在高尔基体中催化PRCD的棕榈酰化。总之，我们发现PRCD的棕榈酰化对其向光感受器OS的运输至关重要，并且该蛋白的错误定位可能导致RP相关的表型。

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