Article

Hepatic mitochondrial respiration is crucial for euthermia in complex III-deficient mice with impaired brown adipose tissue thermogenesis

2024-09-24

Abstract excerpt

Liver is the key hub of systemic energy metabolism and growth regulation, yet its roles in mitochondrial disease pathophysiology remain relatively understudied. Bcs1l p.S78G knock-in mice, carrying a patient mutation causing respiratory complex III (CIII)-deficiency, present juvenile-onset liver and kidney disease, growth restriction, lipodystrophy, and early death. We restored CIII function in the hepatocytes o...

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Literature Corpus work: ea455d0f-b4a3-5909-bf50-f2ad2a8edc7f
DOI: 10.1101/2024.09.23.612616

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