Product Name	MT-ND6
Chinese Name	NADH复合体6抗体
Alias	Mitochondrially encoded NADH dehydrogenase 6; NADH-ubiquinone oxidoreductase chain 6; MT ND6; mtND6; NADH dehydrogenase subunit 6 (complex I); NADH dehydrogenase subunit 6; NADH ubiquinone oxidoreductase chain 6; NADH Ubiquinone Oxidoreductase subunit ND6; NADH6; ND6; NU6M_HUMAN; NU6M_HUMA
Research Area	Tumour  Cell biology  immunology  Neurobiology  transcriptional regulatory factor
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Mouse, Rat,  (predicted: Rabbit, )
Applications	WB=1:500-2000  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	19kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from mouse MT-ND6: 31-130/172
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	NADH Dehydrogenase subunit 6 (MTND6) is 1 of the 7 mitochondrial DNA (mtDNA) encoded subunits (MTND1, MTND2, MTND3, MTND4L, MTND4, MTND5, MTND6) included among the approximately 41 polypeptides of respiratory Complex I. Complex I accepts electrons from NADH, transfers them to ubiquinone (Coenzyme Q10), and uses the energy released to pump protons across the mitochondria inner membrane. MTND6 has been proposed to be a component of the iron-protein fragment. 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 (By similarity). Subcellular Location: Mitochondrion membrane; Multi-pass membrane protein (Potential). DISEASE: Defects in MT-ND6 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. Defects in MT-ND6 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. Defects in MT-ND6 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. Defects in MT-ND6 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. 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. Similarity: Belongs to the complex I subunit 6 family. SWISS: P03923 Gene ID: 4541 Database links: Entrez Gene: 4541 Human Omim: 516006 Human SwissProt: P03923 Human SwissProt: O79882 Pig
Product Picture	Sample: Spleen (Mouse) Lysate at 40 ug Spleen (Rat) Lysate at 40 ug Primary: Anti-MT-ND6 (SL22462R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 24 kD Observed band size: 24 kD

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