Biochemical and neurophysiological effects of deficiency of the mitochondrial import protein TIMM50

Biochemical and neurophysiological effects of deficiency of the mitochondrial import protein TIMM50

TIMM50, an essential TIM23 complex subunit, is suggested to facilitate the import of ~60% of the mitochondrial proteome. In this study, we characterized a TIMM50 disease-causing mutation in human fibroblasts and noted significant decreases in TIM23 core protein levels (TIMM50, TIMM17A/B, and TIMM23)...

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Bibliographic Details
Main Authors: Eyal Paz, Sahil Jain, Irit Gottfried, Orna Staretz-Chacham, Muhammad Mahajnah, Pritha Bagchi, Nicholas T Seyfried, Uri Ashery, Abdussalam Azem
Format: Article
Language:English
Published: eLife Sciences Publications Ltd 2024-12-01
Series:eLife
Subjects:
mitochondria
mitochondrial protein import
TIM23
TIMM50
TIMM50 disease
import disease
Online Access:https://elifesciences.org/articles/99914
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