人 NDUFS5 抗体对
01AB02440
NADH:ubiquinone oxidoreductase subunit S5 (NDUFS5) is a nuclear-encoded accessory subunit of mitochondrial respiratory chain complex I. Localized to the inner mitochondrial membrane, it belongs to the iron-sulfur protein fragment and is essential for the stability and assembly of the complex, although it does not participate directly in electron transfer. The protein contains conserved cysteine motifs that form structural disulfide bonds. NDUFS5 is widely expressed and is vital for maintaining efficient respiratory electron transport. Pathogenic variants in the NDUFS5 gene are associated with mitochondrial complex I deficiency, a heterogeneous disorder that presents with encephalopathy and lactic acidosis. It is also linked to progressive leukoencephalopathy with ovarian failure, reflecting the high energy requirements of neurological and reproductive tissues.
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技术参数
- 靶点基因名
- NDUFS5
- 蛋白别名
- NADH:ubiquinone oxidoreductase subunit S5; NDUFS5
- UniProt
- O43920
- 物种
- Human
产品详情
- 靶点名称
- NDUFS5
- 蛋白名称
- NADH:ubiquinone oxidoreductase subunit S5
- 蛋白别名
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 5; Complex I-15 kDa; CI-15 kDa; NADH-ubiquinone oxidoreductase 15 kDa subunit
- 靶点基因名
- NDUFS5
- UniProt
- O43920
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
NADH:ubiquinone oxidoreductase subunit S5 (NDUFS5) is a nuclear-encoded accessory subunit of mitochondrial respiratory chain complex I. Localized to the inner mitochondrial membrane, it belongs to the iron-sulfur protein fragment and is essential for the stability and assembly of the complex, although it does not participate directly in electron transfer. The protein contains conserved cysteine motifs that form structural disulfide bonds. NDUFS5 is widely expressed and is vital for maintaining efficient respiratory electron transport. Pathogenic variants in the NDUFS5 gene are associated with mitochondrial complex I deficiency, a heterogeneous disorder that presents with encephalopathy and lactic acidosis. It is also linked to progressive leukoencephalopathy with ovarian failure, reflecting the high energy requirements of neurological and reproductive tissues.