抄録
Secretory proteins like plasma proteins are co- or post-translationally processed in many ways on their way of secretion. Many genetic diseases are due to low levels of proteins caused partly by a secretion defect or intracellular degradation. In this review article, such intracellular events of abnormal proteins as a cause of protein deficiency are discussed.
Quality control of newly synthesized protein takes place in the endoplasmic reticulum. Some molecular chaperones are shown to be involved in this quality control mechanism. For example, calnexin, a membrane-bound ER protein is known to function in glucose trimming of plasma glycoproteins. Whether or not the molecular chaperones recognize both normal and abnormal proteins equally is not known yet. But, only abnormal proteins are selectively degraded by some intracellular protease (s), primarily by those of the endoplasmic reticulum.
