Anti-MT-ATP6 antibody [1G7-1G2] - N-terminal (ab219825)

Overview

• Product name
  Anti-MT-ATP6 antibody [1G7-1G2] - N-terminal
  See all MT-ATP6 primary antibodies
• Description
  Mouse monoclonal [1G7-1G2] to MT-ATP6 - N-terminal
• Tested applications
  Suitable for: WBmore details
• Species reactivity
  Reacts with: Human
  
• Immunogen

  Synthetic peptide corresponding to Human MT-ATP6 (N terminal).
  Database link: P00846

• Positive control
  • WB: Mitochondria from cultured normal control human dermal fibroblasts neonatal (HDFn); Whole cell extract of cultured normal control human dermal fibroblasts neonatal (HDFn).

Properties

• Form
  Liquid
• Storage instructions
  Shipped 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 buffer
  pH: 7.20
  Preservative: 0.02% Sodium azide
  Constituents: 0.36% HEPES, 0.87% Sodium chloride
• Concentration information loading...
• Purity
  Protein L purified
• Purification notes
  Purified from hybridoma cell culture supernatant by Protein L affinity chromatography from fetal bovine serum containing medium (Protein L does not bind bovine IgG).
• Clonality
  Monoclonal
• Clone number
  1G7-1G2
• Isotype
  IgG2b
• Light chain type
  kappa

Associated products

• Compatible Secondaries
  • Goat Anti-Mouse IgG H&L (Alexa Fluor® 488) (ab150113)
  • Goat Anti-Mouse IgG H&L (HRP) (ab205719)

Applications

Target

• Function
  Mitochondrial 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 disease
  Defects 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 similarities
  Belongs to the ATPase A chain family.
• Cellular localization
  Mitochondrion inner membrane.

• Target information above from: UniProt accession P00846 The UniProt Consortium
  The Universal Protein Resource (UniProt) in 2010
  Nucleic Acids Res. 38:D142-D148 (2010) .

  Information by UniProt
• Database links
  • Entrez Gene: 4508 Human
  • Omim: 516060 Human
  • SwissProt: P00846 Human
• Alternative names
  • ATP synthase subunit a antibody
  • ATP6 antibody
  • ATP6_HUMAN antibody

  • ATPASE6 antibody
  • F-ATPase protein 6 antibody
  • MT-ATP6 antibody
  • MTATP6 antibody

  see all