ND5 rabbit pAb
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50UL
£ POA
ES2905-50UL
100UL
£ POA
ES2905-100UL
Über dieses Produkt
- SKU:
- ES2905
- Zusätzliche Namen:
- MT-ND5; MTND5; NADH5; ND5; NADH-ubiquinone oxidoreductase chain 5; NADH dehydrogenase subunit 5
- Anwendung:
- Western Blot, ELISA
- Klonalität:
- Polyclonal
- Konzentration:
- 1 mg/ml
- Weitere Details:
- catalytic activity:NADH + ubiquinone = NAD(+) + ubiquinol.;disease:Defects in MT-ND5 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.;disease:Defects in MT-ND5 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-ND5 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.;disease:Defects in MT-ND5 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-ND5 are associated with features of myoclonic epilepsy associated with ragged-red fibers (MERRF) [MIM:545000]. MERRF is a mitochondrial encephalomyopathy characterized by myoclonic seizures. The prevalence in the general population of Europe has been estimated at 0.9 in 100'000 individuals; but the disease seems to be more common in the USA. Patients usually present during adolescence or early adulthood with myoclonic epilepsy; sometimes with neurosensory deafness; optic atrophy; short stature or peripheral neuropathy.;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 5 family.;
- Immunogen:
- The antiserum was produced against synthesized peptide derived from human MT-ND5. AA range:328-377
- Isotyp:
- IgG
- Reaktivitäten:
- Human, Rat, Mouse
- Versandbedingungen:
- Blue Ice
- Quelle:
- Rabbit
- Lagerbedingungen:
- -20[o]C
- Hersteller:
- ELK Biotechnology
- Typ:
- Antibodies: Polyclonal Antibody
