Cyr Lab

Univ of North Carolina Chapel Hill

PHYSIOLOGY

Chapel Hill · United States

NIH-funded
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NIH R01 · 2024

Hsp40 and Hsp70 in Membrane Protein Triage

Project Summary: Membrane protein (MP) biogenesis begins in the endoplasmic reticulum (ER) and is a complex process, as domains on both sides of the ER membrane, and within the membrane bilayer, must fold and assemble. MPs understudy include ABC-Transporters, P-Type ATPases, G-protein coupled receptors, and BRICHOS proteins. Missense mutations that cause misfolding and premature degradation of MPs give rise to diseases such as cystic fibrosis, hypogonadotropic hypogonadism, retinitis pigmentosa and idiopathic lung fibrosis. In order to prevent toxic accumulation, misfolded MPs are targeted for ER-associated degradation (ERAD) by E3 ubiquitin ligase complexes. The ER transmembrane Hsp40…

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