MT-ND6, Antibody

For Research Use Only. Not for use in diagnostic procedures.

Rabbit

1.0mg/ml (lot specific)

Small volumes of anti-MT-ND6 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Western Blot (WB), Immunohistochemistry (IHC)

18,622 Da

mitochondrially encoded NADH dehydrogenase 6

NADH dehydrogenase, subunit 6 (complex I)

NADH-ubiquinone oxidoreductase chain 6

MTND6; NADH6; ND6  [Similar Products]

MT-ND6: 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. Defects in MT-ND6 are a cause of Leber hereditary optic neuropathy (LHON). 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. Defects in MT-ND6 are a cause of Leber hereditary optic neuropathy with dystonia (LDYT); 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. Defects in MT-ND6 are a cause of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS). MELAS is a genetically heterogenious disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness. Defects in MT-ND6 are a cause of mitochondrial complex I deficiency (MT-C1D). A disorder of the mitochondrial respiratory chain that causes a wide range of clinical manifestations from lethal neonatal disease to *****-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. Belongs to the complex I subunit 6 family.

Protein type: EC 1.6.5.3; Energy Metabolism - oxidative phosphorylation; Membrane protein, integral; Membrane protein, multi-pass; Oxidoreductase

Chromosomal Location of Human Ortholog: -

Disease: Leber Optic Atrophy; Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, And Stroke-like Episodes

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