Translation Fidelity and Respiration Deficits in CLPP-Deficient Tissues: Mechanistic Insights from Mitochondrial Complexome Profiling

Translation Fidelity and Respiration Deficits in CLPP-Deficient Tissues: Mechanistic Insights from Mitochondrial Complexome Profiling

The mitochondrial matrix peptidase CLPP is crucial during cell stress. Its loss causes Perrault syndrome type 3 (PRLTS3) with infertility, neurodegeneration, and a growth deficit. Its target proteins are disaggregated by CLPX, which also regulates heme biosynthesis via unfolding ALAS enzymes, provid...

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Bibliographic Details
Main Authors:	Jana Key, Suzana Gispert, Gabriele Koepf, Julia Steinhoff-Wagner, Marina Reichlmeir, Georg Auburger
Format:	Article
Language:	English
Published:	MDPI AG 2023-12-01
Series:	International Journal of Molecular Sciences
Subjects:	primary ovarian insufficiency azoospermia sensorineural hearing loss mtLSU quality control rRNA chaperone ERAL1
Online Access:	https://www.mdpi.com/1422-0067/24/24/17503

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