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Assistance for Folding of Disease-Causing Plasma Membrane Proteins.

ABSTRACT: 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.

SUBMITTER: Juarez-Navarro K

PROVIDER: S-EPMC7277483 | biostudies-literature | 2020 May

REPOSITORIES: biostudies-literature

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Assistance for Folding of Disease-Causing Plasma Membrane Proteins.

Juarez-Navarro Karina K Ayala-Garcia Victor M VM Ruiz-Baca Estela E Meneses-Morales Ivan I Rios-Banuelos Jose Luis JL Lopez-Rodriguez Angelica A

Biomolecules 20200507 5

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 patholog ...[more]

PMID: 32392767

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