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Coatopathies: Genetic Disorders of Protein Coats

Esteban C Dell'Angelica, Juan S Bonifacino

Annu Rev Cell Dev Biol. 2019 Oct 6;35:131-168.

PMID: 31399000

Abstract:

Protein coats are supramolecular complexes that assemble on the cytosolic face of membranes to promote cargo sorting and transport carrier formation in the endomembrane system of eukaryotic cells. Several types of protein coats have been described, including COPI, COPII, AP-1, AP-2, AP-3, AP-4, AP-5, and retromer, which operate at different stages of the endomembrane system. Defects in these coats impair specific transport pathways, compromising the function and viability of the cells. In humans, mutations in subunits of these coats cause various congenital diseases that are collectively referred to as coatopathies. In this article, we review the fundamental properties of protein coats and the diseases that result from mutation of their constituent subunits.

Chemicals Related in the Paper:

Catalog Number Product Name Structure CAS Number Price
AP143621356 3-AP 3-AP 143621-35-6 Price
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