Anti-MT-ATP6 antibody - C-terminal (ab190287)

Overview

  • Product name

    Anti-MT-ATP6 antibody - C-terminal
    See all MT-ATP6 primary antibodies
  • Description

    Goat polyclonal to MT-ATP6 - C-terminal
  • Host species

    Goat
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Synthetic peptide within Human MT-ATP6 aa 32-46 (C terminal) (Cysteine residue). The exact sequence is proprietary. YP_003024031.1
    Sequence:

    PTSKYLINNRLITTQ


    Database link: P00846

  • Positive control

    • Human heart lysate

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: 7.30
    Preservative: 0.02% Sodium azide
    Constituents: 0.5% BSA, 99% Tris buffered saline
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Purification notes

    ab190287 was purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
  • Clonality

    Polyclonal
  • Isotype

    IgG

Applications

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.

Application Abreviews Notes
WB Use a concentration of 0.1 - 0.3 µg/ml. Detects a band of approximately 26 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

  • 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

References

ab190287 has not yet been referenced specifically in any publications.

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