Assistance for Folding of Disease-Causing Plasma Membrane Proteins

Biomolecules

Karina Juarez-Navarro¹  VictorM. Ayala-Garcia¹  JoseLuis Rios-Banuelos¹  Angelica Lopez-Rodriguez¹  Estela Ruiz-Baca¹  Ivan Meneses-Morales¹ 

[1] Facultad de Ciencias Quimicas, Universidad Juarez del Estado de Durango, Durango C.P 34000, Mexico;

关键词: proteostasis;    quality control;    misrouting;    chaperones;   

DOI  :  10.3390/biom10050728

来源: DOAJ

【 摘 要 】

An extensive catalog of plasma membrane (PM) protein mutations related to phenotypic diseases is associated with incorrect protein folding and/or localization. These impairments, in addition to dysfunction, frequently promote protein aggregation, which can be detrimental to cells. Here, we review PM protein processing, from protein synthesis in the endoplasmic reticulum to delivery to the PM, stressing the main repercussions of processing failures and their physiological consequences in pathologies, and we summarize the recent proposed therapeutic strategies to rescue misassembled proteins through different types of chaperones and/or small molecule drugs that safeguard protein quality control and regulate proteostasis.

【 授权许可】

Unknown