产品名称
NU4M Rabbit Polyclonal Antibody
基因名称
MT-ND4 MTND4 NADH4 ND4
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/4538
Human Swissprot No.
P03905
Human Swissprot Link
https://www.uniprot.org/uniprotkb/P03905/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/17719
Mouse Swissprot No.
P03911
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/P03911
Rat Swissprot Link
https://www.uniprot.org/uniprotkb/P05508
免疫原
Synthesized peptide derived from human NU4M AA range: 281-331
特异性
This antibody detects endogenous levels of NU4M at Human/Mouse/Rat
宿主
Polyclonal, Rabbit,IgG
细胞定位
Mitochondrion inner membrane ; Multi-pass membrane protein .
功能
catalytic activity:NADH + ubiquinone = NAD(+) + ubiquinol.,disease:Defects in MT-ND4 are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.,disease:Defects in MT-ND4 are a cause of Leber hereditary optic neuropathy with dystonia (LDYT) [MIM:500001]; also called familial dystonia with visual failure and striatal lucencies. LDYT is part of a spectrum of Leber hereditary optic neuropathy. It is characterized by the association of optic atrophy and central vision loss with dystonia.,disease:Defects in MT-ND4 are a cause of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS) [MIM:540000]. MELAS is a genetically heterogenious disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness.,disease:Defects in MT-ND4L are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.,function:Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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 subunit 4 family.,similarity:Belongs to the complex I subunit 4L family.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.