ANTI-MT-ATP6 CLONE 1G7-1G2 (C15-1885-489)

General description

ATP synthase subunit a (UniProt: P00846; also known as F-ATPase protein 6) is encoded by the MT-ATP6 (also known as ATP6, ATPASE6, MTATP6) gene (Gene ID: 4508) in human. F-ATPase Protein 6 is an inner mitochondrial membrane protein and is a component of Complex V, which produces ATP from ADP in the presence of proton gradient across the membrane. F-type ATPases have 2 components, CF1 - the catalytic core - and CF0- the membrane proton channel. The CF1 catalytic core contains five subunits: alpha3, beta3, gamma1, delta1, epsilon1 and CF0 has three main subunits known as a, b, and c. Together they form a rotary motor. During catalysis, ATP synthesis in the catalytic domain of F1 is coupled via a rotary mechanism of the central stalk subunits to proton translocation. Defects in MT-ATP6 gene cause multiple mitochondrial diseases, such as Leber hereditary optic neuropathy (LHON), which is characterized by acute and subacute loss of central vision due to optic nerve dysfunction. Some defects also lead to mitochondrial complex V deficiency that leads to neuropathy, ataxia, and hypertrophic cardiomyopathy.

Specificity

Clone 1G7-1G2 detects ATP synthase subunit a (MT-ATP6) in mitochondria isolated from human cells.

Immunogen

A synthetic peptide corresponding to the N-terminus of human MT-ATP6.

Epitope: N-terminus

Application

Anti-MT-ATP6, clone 1G7-1G2, Cat. No. MABS1995, is a mouse monoclonal antibody that detects ATP synthase subunit a and has been tested for use in Western Blotting.

Research Category
Signaling

Western Blotting Analysis: 0.5 µg/mL from a representative lot detected MT-ATP6 in mitochondria from human neonatal dermal fibroblasts and mitochondria from human neonatal dermal fibroblasts depleted of mtDNA (Courtesy of Michael F. Marusich, Ph.D., mAbDx, Inc., Eugene, OR USA).

Quality

Evaluated by Western Blotting in Mitochondria from human neonatal dermal fibroblasts and mitochondria from human neonatal dermal fibroblasts depleted of mtDNA.

Western Blotting Analysis: 1 µg/mL of this antibody detected MT-ATP6 in Mitochondria from human neonatal dermal fibroblasts and mitochondria from human neonatal dermal fibroblasts depleted of mtDNA.

Target description

~20 kDa observed; 24.82 kDa calculated. Uncharacterized bands may be observed in some lysate(s).

Physical form

Format: Purified

Protein L

Purified mouse monoclonal antibody IgG2b in HEPES-Buffered Saline (15 mM HEPES, 150 mM NaCl, pH 7.2) with 0.02% sodium azide.

Storage and Stability

Stable for 1 year at 2-8°C from date of receipt.

Other Notes

Concentration: Please refer to lot specific datasheet.

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.