抗人 NDUFS4 抗体对
01AB03340
NADH:ubiquinone oxidoreductase subunit S4 (NDUFS4) is a nuclear-encoded accessory subunit of mitochondrial Complex I. While not directly catalytic, it is indispensable for the proper assembly, stability, and activity of the complex, facilitating electron transfer from NADH to ubiquinone in the respiratory chain. NDUFS4 is essential for mitochondrial oxidative phosphorylation, particularly in tissues with high energy demand like the brain and muscle. Pathogenic variants in NDUFS4 disrupt Complex I function and are a frequent cause of Leigh syndrome, a severe early-onset neurodegenerative disorder characterized by lactic acidosis, psychomotor regression, and brainstem lesions.
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技术参数
- 靶点基因名
- NDUFS4
- 蛋白别名
- NADH:ubiquinone oxidoreductase subunit S4; NDUFS4
- UniProt
- O43181
- 物种
- Human
产品详情
- 靶点名称
- NDUFS4
- 蛋白名称
- NADH:ubiquinone oxidoreductase subunit S4
- 蛋白别名
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 4; mitochondrial; Complex I-18 kDa; CI-18 kDa; Complex I-AQDQ; CI-AQDQ; NADH-ubiquinone oxidoreductase 18 kDa subunit
- 靶点基因名
- NDUFS4
- UniProt
- O43181
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
NADH:ubiquinone oxidoreductase subunit S4 (NDUFS4) is a nuclear-encoded accessory subunit of mitochondrial Complex I. While not directly catalytic, it is indispensable for the proper assembly, stability, and activity of the complex, facilitating electron transfer from NADH to ubiquinone in the respiratory chain. NDUFS4 is essential for mitochondrial oxidative phosphorylation, particularly in tissues with high energy demand like the brain and muscle. Pathogenic variants in NDUFS4 disrupt Complex I function and are a frequent cause of Leigh syndrome, a severe early-onset neurodegenerative disorder characterized by lactic acidosis, psychomotor regression, and brainstem lesions.