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

Applications

Our Abpromise guarantee covers the use of ab219825 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 a concentration of 0.5 - 5 µg/ml. Detects a band of approximately 22 kDa (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 [1G7-1G2] - N-terminal (ab219825) at 0.5 µg/ml

    Lane 1 : Mitochondria from cultured normal control human dermal fibroblasts neonatal (HDFn)
    Lane 2 : Mitochondria from HDFn cells depleted of mtDNA by long-term proliferation in the presence of ethidium bromide

    Lysates/proteins at 10 µg per lane.

    Secondary
    HRP-labeled Goat-anti-mouse IgG
    Developed using the ECL technique

    Predicted band size : 25 kDa
    Observed band size : 22 kDa (why is the actual band size different from the predicted?)

    Mitochondrial proteins solubilized in 2% SDS were separated by SDS-PAGE and then transferred to PVDF membranes in CAPS buffer.

  • All lanes : Anti-MT-ATP6 antibody [1G7-1G2] - N-terminal (ab219825) at 5 µg/ml

    Lane 1 : Whole cell extract of cultured normal control human dermal fibroblasts neonatal (HDFn)
    Lane 2 : Whole cell extract HDFn-Rho0 cells depleted of mtDNA by long-term culture in the presence of ethidium bromide

    Lysates/proteins at 15 µg per lane.

    Secondary
    HRP-labeled Goat-anti-mouse IgG
    Developed using the ECL technique

    Predicted band size : 25 kDa
    Observed band size : 22 kDa (why is the actual band size different from the predicted?)

References

ab219825 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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