Anti-CHCHD10 antibody [EPR26966-13] - BSA and Azide free (Capture)

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

The CHCHD10 protein also known as coiled-coil-helix-coiled-coil-helix domain containing 10 is a mitochondrial protein with a molecular weight of approximately 14 kDa. It is specifically localized within the intermembrane space of mitochondria and is expressed in various tissues with high levels in the brain heart and skeletal muscles. CHCHD10 plays a role in maintaining mitochondrial structure and function contributing to the stability and regulation of mitochondrial cristae.

Biological function summary

The CHCHD10 protein participates in mitochondrial processes that regulate cellular energy production and apoptosis. It is part of a complex network within mitochondria interacting with other proteins to support mitochondrial DNA maintenance and oxidative phosphorylation. Its presence helps maintain cellular homeostasis by preventing mitochondrial dysfunction which can otherwise trigger oxidative stress and cellular damage.

Pathways

CHCHD10 is integral in the maintenance of mitochondrial quality control and respiratory pathways. It is involved in the mitochondrial inner membrane organizing system (MINOS) complex that is important for organizing and stabilizing mitochondrial membrane structure. CHCHD10 also interacts with proteins like OPA1 and CHCHD2 which are important for mitochondrial dynamics and energy regulation demonstrating its key role in these pathways.

Associated diseases and disorders

CHCHD10 mutations are linked to conditions like amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). The interaction between CHCHD10 and other mitochondria-related proteins like SOD1 and TDP-43 highlights its role in the pathogenesis of these neurodegenerative diseases. These mutations lead to mitochondrial dysfunction which contributes to the degeneration of motor neurons in ALS and the degeneration of frontal and temporal lobes in FTD.