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首页> 外文期刊>Pigment cell research >The ocular albinism type 1 (OA1) protein and the evidence for an intracellular signal transduction system involved in melanosome biogenesis
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The ocular albinism type 1 (OA1) protein and the evidence for an intracellular signal transduction system involved in melanosome biogenesis

机译：眼白化病1型（OA1）蛋白和涉及黑素体生物发生的细胞内信号转导系统的证据

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摘要

Ocular albinism type 1 is an X-linked disorder characterized by severe reduction of visual acuity, retinal hypopigmentation, foveal hypoplasia, optic misrouting and the presence of giant melanosomes (macromelanosomes) in skin melanocytes and retinal pigment epithelium. The protein product of the OA1 gene is a pigment cell specific membrane glycoprotein, displaying structural and functional features of G protein-coupled receptors (GPCRs). However, in contrast to all other previously characterized GPCRs, OA1 is not localized to the plasma membrane, but is targeted to intracellular organelles, namely late endosomes/lysosomes and melanosomes. These unique characteristics suggest that OA1 represents the first example described so far of an exclusively intracellular GPCR and regulates melanosome biogenesis by transducing signals from the organelle lumen to the cytosol. These findings support previous hypotheses that GPCR-mediated signaling might also operate at the internal membranes in mammalian cells.
机译：1型白化病是一种X连锁疾病，其特征是视力严重降低，视网膜色素沉着，中央凹发育不全，视神经错觉以及皮肤黑素细胞和视网膜色素上皮细胞中存在巨大的黑素体（宏黑素体）。 OA1基因的蛋白质产物是色素细胞特异性膜糖蛋白，显示出G蛋白偶联受体（GPCR）的结构和功能特征。然而，与所有其他先前表征的GPCR相比，OA1并不局限于质膜，而是靶向细胞内的细胞器，即晚期的内体/溶酶体和黑素体。这些独特的特征表明，OA1代表了迄今为止唯一的细胞内GPCR的第一个例子，并通过将信号从细胞器内腔转导至细胞质来调节黑素体的生物发生。这些发现支持以前的假设，即GPCR介导的信号转导也可能在哺乳动物细胞的内膜上起作用。

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