Key features and details
- Rabbit polyclonal to MT-ATP6
- Suitable for: WB
- Reacts with: Mouse, Rat, Human
- Isotype: IgG
Product nameAnti-MT-ATP6 antibody
See all MT-ATP6 primary antibodies
DescriptionRabbit polyclonal to MT-ATP6
SpecificityDetects endogenous levels of MT-ATP6 protein.
Tested applicationsSuitable for: WBmore details
Species reactivityReacts with: Mouse, Rat, Human
Synthetic peptide corresponding to Human MT-ATP6.
Database link: P00846
- HeLa, A549 and PC12 whole cell lysates.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferPreservative: 0.1% Sodium azide
Constituents: 50% Glycerol, 49% PBS
Concentration information loading...
PurityImmunogen affinity purified
Purification notesab192423 was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Our Abpromise guarantee covers the use of ab192423 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use at an assay dependent concentration. Predicted molecular weight: 25 kDa.|
FunctionMitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the central stalk subunits to proton translocation. Key component of the proton channel; it may play a direct role in the translocation of protons across the membrane.
Involvement in diseaseDefects in MT-ATP6 are the cause of neurogenic muscle weakness, ataxia, and retinitis pigmentosa (NARP) [MIM:551500].
Defects in MT-ATP6 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-ATP6 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.
Defects in MT-ATP6 are a cause of mitochondrial infantile bilateral striatal necrosis (MIBSN) [MIM:500003]. Bilateral striatal necrosis is a neurological disorder resembling Leigh syndrome.
Sequence similaritiesBelongs to the ATPase A chain family.
Cellular localizationMitochondrion inner membrane.
- Information by UniProt
- ATP synthase subunit a antibody
- ATP6 antibody
- ATP6_HUMAN antibody
ab192423 has been referenced in 4 publications.
- Zhu Z et al. Rapamycin-mediated mTOR inhibition impairs silencing of sex chromosomes and the pachytene piRNA pathway in the mouse testis. Aging (Albany NY) 11:185-208 (2019). PubMed: 30636722
- Grover R et al. Myg1 exonuclease couples the nuclear and mitochondrial translational programs through RNA processing. Nucleic Acids Res 47:5852-5866 (2019). PubMed: 31081026
- Spangenberg L et al. Deep sequencing discovery of causal mtDNA mutations in a patient with unspecific neurological disease. Mitochondrion 46:337-344 (2019). PubMed: 30227252
- Maio N et al. A Single Adaptable Cochaperone-Scaffold Complex Delivers Nascent Iron-Sulfur Clusters to Mammalian Respiratory Chain Complexes I-III. Cell Metab 25:945-953.e6 (2017). PubMed: 28380382