Overview

  • Product name
  • Description
    Rabbit polyclonal to MT-ATP6
  • Specificity
    Detects endogenous levels of MT-ATP6 protein.
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide corresponding to Human MT-ATP6.
    Database link: P00846

  • Positive control
    • HeLa, A549 and PC12 whole cell lysates.

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
    Preservative: 0.1% Sodium azide
    Constituents: 50% Glycerol, 49% PBS
  • Concentration information loading...
  • Purity
    Immunogen affinity purified
  • Purification notes
    ab192423 was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
  • Clonality
    Polyclonal
  • Isotype
    IgG

Applications

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.

Application Abreviews Notes
WB Use at an assay dependent concentration. Predicted molecular weight: 25 kDa.

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.
  • Information by UniProt
  • Database links
  • 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

Images

  • All lanes : Anti-MT-ATP6 antibody (ab192423)

    Lane 1 : HeLa whole cell lysate
    Lane 2 : A549 whole cell lysate
    Lane 3 : PC12 whole cell lysate


    Predicted band size : 25 kDa

References

ab192423 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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