Neurodegeneration-associated mitochondrial proteins, CHCHD2 and CHCHD10-what distinguishes the two?

Aya Ikeda; Yuzuru Imai; Nobutaka Hattori

doi:10.3389/fcell.2022.996061

Neurodegeneration-associated mitochondrial proteins, CHCHD2 and CHCHD10-what distinguishes the two?

Front Cell Dev Biol. 2022 Sep 9:10:996061. doi: 10.3389/fcell.2022.996061. eCollection 2022.

Authors

Aya Ikeda¹, Yuzuru Imai^{1

2}, Nobutaka Hattori^{1

2

3

4

5}

Affiliations

¹ Department of Neurology, Juntendo University School of Medicine, Tokyo, Japan.
² Department of Research for Parkinson's Disease, Juntendo University Graduate School of Medicine, Tokyo, Japan.
³ Research Institute for Diseases of Old Age, Graduate School of Medicine, Juntendo University, Tokyo, Japan.
⁴ Center for Genomic and Regenerative Medicine, Graduate School of Medicine, Juntendo University, Tokyo, Japan.
⁵ Neurodegenerative Disorders Collaborative Laboratory, RIKEN Center for Brain Science, Saitama, Japan.

Abstract

Coiled-coil-helix-coiled-coil-helix domain containing 2 (CHCHD2) and Coiled-coil-helix-coiled-coil-helix domain containing 10 (CHCHD10) are mitochondrial proteins that are thought to be genes which duplicated during evolution and are the causative genes for Parkinson's disease and amyotrophic lateral sclerosis/frontotemporal lobe dementia, respectively. CHCHD2 forms a heterodimer with CHCHD10 and a homodimer with itself, both of which work together within the mitochondria. Various pathogenic and disease-risk variants have been identified; however, how these mutations cause neurodegeneration in specific diseases remains a mystery. This review focuses on important new findings published since 2019 and discusses avenues to solve this mystery.

Keywords: amyothophic lateral sclerosis; dopaminergic (DA) neuron; genetics; mitochondria; motor neurons; parkinson's disease.

Publication types

Review