Rabbit anti-Human MT-ND6 Polyclonal Antibody | anti-MT-ND6 antibody

MT-ND6 Conjugated Antibody

Cat. Num. AAA9448180

• Gene Names: MTBP; MDM2BP
• Reactivity: Human
• Applications: Most Applications
• Synonyms: MT-ND6; Polyclonal Antibody; MT-ND6 Conjugated Antibody; MTND6; ND6; anti-MT-ND6 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous levels of total MT-ND6 protein.
• Form/Format: 0.01M Sodium Phosphate, 0.25M NaCl, pH 7.6, 5mg/ml Bovine Serum Albumin, 0.02% Sodium Azide

• Applicable Applications for anti-MT-ND6 antibody: Most Applications
• Application Notes: Suggested Dilution:; AF350 conjugated: most applications: 1:50-1:250; AF405 conjugated: most applications: 1:50-1:250; AF488 conjugated: most applications: 1:50-1:250; AF555 conjugated: most applications: 1:50-1:250; AF594 conjugated: most applications: 1:50-1:250; AF647 conjugated: most applications: 1:50-1:250; AF680 conjugated: most applications: 1:50-1:250; AF750 conjugated: most applications: 1:50-1:250; Biotin conjugated: working with enzyme-conjugated streptavidin, most applications: 1:50-1:1000
• Immunogen: Synthetic peptide corresponding to a region derived from internal residues of human mitochondrially encoded NADH dehydrogenase 6
• Conjugation: Biotin AF350 AF405 AF488 AF555 AF594 AF647 AF680 AF750
• Excitation Emission: AF350: 346nm/442nm
• Preparation and Storage: Store at 4 degree C for 6 months.

Related Product Information for anti-MT-ND6 antibody

NADH:ubiquinone oxidoreductase (complex I) is an extremely complicated multiprotein complex located in the inner mitochondrial membrane. Human complex I is important for energy metabolism because its main function is to transport electrons from NADH to ubiquinone, which is accompanied by translocation of protons from the mitochondrial matrix to the intermembrane space. Human complex I appears to consist of 41 subunits. A small number of complex I subunits are the products of mitochondrial genes (subunits 1-7), while the remainder are nuclear encoded and imported from the cytoplasm. The significance of NADH dehydrogenase subunit 6 (ND6) is rapidly becoming increasingly apparent as many mutations leading to amino acid changes in this subunit are associated with known mitochondrial diseases.

NCBI and Uniprot Product Information

• NCBI GI #: 21630257
• NCBI GeneID: 27085
• NCBI Accession #: NP_071328
• NCBI GenBank Nucleotide #: NM_022045.4
• UniProt Accession #: P03923; Q9HA89; B4DUR5; Q96DY7
• Molecular Weight: 104 kDa
• NCBI Official Full Name: mdm2-binding protein
• NCBI Official Synonym Full Names: MDM2 binding protein
• NCBI Official Symbol: MTBP
• NCBI Official Synonym Symbols: MDM2BP
• NCBI Protein Information: mdm2-binding protein; MDM2 (mouse double minute 2)-binding protein, 104kD; Mdm2, transformed 3T3 cell double minute 2, p53 binding protein binding protein, 104kDa
• UniProt Protein Name: NADH-ubiquinone oxidoreductase chain 6
• Protein Family: NADH-ubiquinone oxidoreductase
• UniProt Gene Name: MT-ND6
• UniProt Synonym Gene Names: MTND6; NADH6; ND6
• UniProt Entry Name: NU6M_HUMAN

NCBI Description

This gene encodes a protein that interacts with the oncoprotein mouse double minute 2. The encoded protein regulates progression through the cell cycle and may be involved in tumor formation. [provided by RefSeq, Aug 2012]

Uniprot Description

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

Chromosomal Location of Human Ortholog: -

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