NADH Reductive Stress and Its Correlation with Disease Severity in Leigh Syndrome: A Pilot Study Using Patient Fibroblasts and a Mouse Model

NADH Reductive Stress and Its Correlation with Disease Severity in Leigh Syndrome: A Pilot Study Using Patient Fibroblasts and a Mouse Model

Nicotinamide adenine dinucleotide (NAD) is a critical cofactor in mitochondrial energy production. The NADH/NAD<sup>+</sup> ratio, reflecting the balance between NADH (reduced) and NAD<sup>+ (</sup>oxidized<sup>)</sup>, is a key marker for the severity of mitochon...

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Main Authors: Tamaki Ishima, Natsuka Kimura, Mizuki Kobayashi, Chika Watanabe, Eriko F. Jimbo, Ryosuke Kobayashi, Takuro Horii, Izuho Hatada, Kei Murayama, Akira Ohtake, Ryozo Nagai, Hitoshi Osaka, Kenichi Aizawa
Format: Article
Language:English
Published: MDPI AG 2024-12-01
Series:Biomolecules
Subjects:
LC-MS/MS
NADH
reductive stress
Leigh syndrome
mitochondrial diseases
<i>Ndufs4</i>-KO mice
Online Access:https://www.mdpi.com/2218-273X/15/1/38
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