产品名称
NDUS4 Rabbit Polyclonal Antibody
蛋白名称
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)
存储缓冲液
Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.
Human Swissprot No.
O43181
Human Swissprot Link
https://www.uniprot.org/uniprotkb/O43181/entry
Mouse Swissprot No.
Q9CXZ1
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q9CXZ1
Rat Swissprot Link
http://www.uniprot.org/uniprot/O54941Q5XIF3
免疫原
Synthesized peptide derived from human protein . at AA range: 20-100
特异性
NDUS4 Polyclonal Antibody detects endogenous levels of protein.
稀释度
WB 1:500-2000 ELISA 1:5000-20000
宿主
Polyclonal, Rabbit,IgG
背景介绍
This gene encodes an nuclear-encoded accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I, or NADH:ubiquinone oxidoreductase). Complex I removes electrons from NADH and passes them to the electron acceptor ubiquinone. Mutations in this gene can cause mitochondrial complex I deficiencies such as Leigh syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2015],
组织表达
T-cell,Urinary bladder,
细胞定位
Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side . The interaction with BCAP31 mediates mitochondria localization. .
信号通路
Oxidative phosphorylation;Alzheimer's disease;Parkinson's disease;Huntington's disease;
功能
disease:Defects in NDUFS4 are a cause of complex I mitochondrial respiratory chain deficiency [MIM:252010]. Complex I (NADH-ubiquinone oxidoreductase), the largest complex of the mitochondrial respiratory chain, contains more than 40 subunits. It is embedded in the inner mitochondrial membrane and is partly protruding in the matrix. Complex I deficiency is the most common cause of mitochondrial disorders. It represents largely one-third of all cases of respiratory chain deficiency and is responsible for a variety of clinical symptoms, ranging from neurological disorders to cardiomyopathy, liver failure, and myopathy.,function:Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed to be not involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.,similarity:Belongs to the complex I NDUFS4 subunit family.,subunit:Mammalian complex I is composed of 45 different subunits. This is a component of the iron-sulfur (IP) fragment of the enzyme.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.