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 bufferpH: 7.30
Preservative: 0.02% Sodium azide
Constituents: 0.5% BSA, 99% Tris buffered saline
Concentration information loading...
PurityImmunogen affinity purified
Purification notesab190287 was purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Our Abpromise guarantee covers the use of ab190287 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 0.1 - 0.3 µg/ml. Detects a band of approximately 26 kDa (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
Anti-MT-ATP6 antibody - C-terminal (ab190287) at 0.1 µg/ml + Human heart lysate at 35 µg
Developed using the ECL technique.
Predicted band size: 25 kDa
Observed band size: 26 kDa why is the actual band size different from the predicted?
Exposure time: 1 hour
ab190287 has not yet been referenced specifically in any publications.