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Updated: Sep 10, 2025

Author Spotlight: Advancing Techniques and Discoveries in Protein Synthesis and Assembly Through Innovative Mitochondrial Research
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COXPD8における機能障害のある電子輸送連鎖組成

Gisela Beutner1, Heidie L Huyck2, Gail Deutsch3

  • 1Department of Pediatrics-Division Cardiology, University of Rochester Medical Center, Rochester, NY 14642, USA.

Journal of cardiovascular development and disease
|August 27, 2025
PubMed
まとめ

AARS2遺伝子変異によって引き起こされる8型酸化リン酸化欠乏症 (COXPD8) は,重度の乳児疾患を引き起こす. これらの変異は電子輸送鎖の複合組成と超複合体の形成を阻害し,バイオエネルギーストレスを引き起こします.

キーワード:
COXPD8 について電子輸送連鎖ハイパルトロフィック心筋病ミトコンドリア疾患ミトコンドリア超複合体

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