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首页> 外文期刊>Physiological Reviews >In and out of the ER: protein folding, quality control, degradation, and related human diseases.
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In and out of the ER: protein folding, quality control, degradation, and related human diseases.

机译：进入和退出ER：蛋白质折叠，质量控制，降解和相关的人类疾病。

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摘要

A substantial fraction of eukaryotic gene products are synthesized by ribosomes attached at the cytosolic face of the endoplasmic reticulum (ER) membrane. These polypeptides enter cotranslationally in the ER lumen, which contains resident molecular chaperones and folding factors that assist their maturation. Native proteins are released from the ER lumen and are transported through the secretory pathway to their final intra- or extracellular destination. Folding-defective polypeptides are exported across the ER membrane into the cytosol and destroyed. Cellular and organismal homeostasis relies on a balanced activity of the ER folding, quality control, and degradation machineries as shown by the dozens of human diseases related to defective maturation or disposal of individual polypeptides generated in the ER.
机译：大部分真核基因产物是由附着在内质网（ER）膜胞质面上的核糖体合成的。这些多肽共翻译进入ER内腔，其中包含常驻分子伴侣和有助于其成熟的折叠因子。天然蛋白从ER内腔释放，并通过分泌途径转运至其最终的细胞内或细胞外目的地。折叠缺陷的多肽通过ER膜进入细胞质并被破坏。细胞和生物体内稳态依赖于ER折叠，质量控制和降解机制的平衡活动，如与ER产生的单个多肽的成熟缺陷或处置不良有关的数十种人类疾病所示。

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