MT-ND5 Antikörper (AA 328-377)

Produktnummer ABIN1535072

Der Kaninchen Polyklonal anti-MT-ND5 Antikörper (ABIN1535072) detektiert spezifisch MT-ND5 in WB und ELISA. Dieser Antikörper reagiert spezifisch mit Proben aus Human, Ratte und Maus.

Kurzübersicht für MT-ND5 Antikörper (AA 328-377) (ABIN1535072)

Target: MT-ND5 (Mitochondrially Encoded NADH Dehydrogenase 5 (MT-ND5))

Reaktivität: Human, Ratte, Maus

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser MT-ND5 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA

Produktdetails anti-MT-ND5 Antikörper

Bindungsspezifität: AA 328-377

Verwendungszweck: ND5 Antibody

Spezifität: ND5 Polyclonal Antibody detects endogenous levels of ND5 protein.

Aufreinigung: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Reinheit: > 95 %

Immunogen: The antiserum was produced against synthesized peptide derived from human MT-ND5. AA range:328-377

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 mg/mL

Buffer: Liquid in PBS containing 50 % glycerol, 0.5 % BSA and 0.02 % sodium azide.

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C/1 year

Haltbarkeit: 12 months

Details zu MT-ND5

Target: MT-ND5 (Mitochondrially Encoded NADH Dehydrogenase 5 (MT-ND5))

Andere Bezeichnung: MT-ND5

Hintergrund:

Synonyms: MT-ND5, MTND5, NADH5, ND5, NADH-ubiquinone oxidoreductase chain 5, NADH dehydrogenase subunit 5

Background: 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.,

Gene Name: MT-ND5

Protein Name: NADH-ubiquinone oxidoreductase chain 5

Molekulargewicht: 67 kDa

Gen-ID: 4540

OMIM: 252010

UniProt: P03915