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Twin CHCH Proteins, CHCHD2, and CHCHD10: Key Molecules of Parkinson’s Disease, Amyotrophic Lateral Sclerosis, and Frontotemporal Dementia

1
Department of Research for Parkinson’s Disease, Juntendo University Graduate School of Medicine, Tokyo 113-8421, Japan
2
Department of Treatment and Research in Multiple Sclerosis and Neuro-intractable Disease, Juntendo University Graduate School of Medicine, Tokyo 113-8421, Japan
3
Department of Neurology, Juntendo University Graduate School of Medicine, Tokyo 113-8421, Japan
4
Department of Neurodegenerative and Demented Disorders, Juntendo University Graduate School of Medicine, Tokyo 113-8421, Japan
*
Authors to whom correspondence should be addressed.
Int. J. Mol. Sci. 2019, 20(4), 908; https://doi.org/10.3390/ijms20040908
Received: 7 February 2019 / Revised: 15 February 2019 / Accepted: 17 February 2019 / Published: 20 February 2019
Mutations of coiled-coil-helix-coiled-coil-helix domain containing 2 (CHCHD2) and 10 (CHCHD10) have been found to be linked to Parkinson’s disease (PD), amyotrophic lateral sclerosis (ALS), and/or frontotemporal lobe dementia (FTD). CHCHD2 and CHCHD10 proteins, which are homologous proteins with 54% identity in amino acid sequence, belong to the mitochondrial coiled-coil-helix-coiled-coil-helix (CHCH) domain protein family. A series of studies reveals that these twin proteins form a multimodal complex, producing a variety of pathophysiology by the disease-causing variants of these proteins. In this review, we summarize the present knowledge about the physiological and pathological roles of twin proteins, CHCHD2 and CHCHD10, in neurodegenerative diseases. View Full-Text
Keywords: coiled-coil-helix-coiled-coil-helix domain; OXPHOS; respiratory complexes; mitochondria; CHCH domain; MICOS; neurodegenerative diseases coiled-coil-helix-coiled-coil-helix domain; OXPHOS; respiratory complexes; mitochondria; CHCH domain; MICOS; neurodegenerative diseases
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MDPI and ACS Style

Imai, Y.; Meng, H.; Shiba-Fukushima, K.; Hattori, N. Twin CHCH Proteins, CHCHD2, and CHCHD10: Key Molecules of Parkinson’s Disease, Amyotrophic Lateral Sclerosis, and Frontotemporal Dementia. Int. J. Mol. Sci. 2019, 20, 908.

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