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Anti-MT-ATP6 antibody (ab102573)抗線粒體MT-ATP6抗體產品信息：

• Product nameAnti-MT-ATP6 antibodySee all MT-ATP6 primary antibodies ...
• Description

  Rabbit polyclonal to MT-ATP6

• Tested applicationsICC/IF, WB more details
• Species reactivity

  Reacts with: Human

• Immunogen

  Synthetic peptide conjugated to KLH derived from within residues 1 - 100 of Human MT-ATP6.

  Read Abcam's proprietary immunogen policy

   

   

• Positive control
  • This antibody gave a positive signal in Human Skin tissue lysate.

Properties

• FormLiquid
• Storage instructionsStore at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
• Storage bufferpH: 7.40
  Preservative: 0.02% Sodium azide
  Constituent: PBS
  Note: Batches of this product that have a concentration < 1mg/ml may have BSA added as a stabilising agent. If you would like information about the formulation of a specific lot, please contact our scientific support team who will be happy to help.

• Concentration 200 µl at 0.1 mg/ml

• PurityImmunogen affinity purified
• Clonality Polyclonal
• IsotypeIgG

Applications

Our Abpromise guarantee covers the use of ab102573 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Target

• FunctionMitochondrial 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 diseaseDefects 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 similaritiesBelongs to the ATPase A chain family.
• Cellular localizationMitochondrion inner membrane.
• Information by UniProt

• Database links

  • Entrez Gene: 4508 Human
  • SwissProt: P00846 Human

• Alternative names

  • ATP synthase subunit a antibody
  • ATP6 antibody
  • ATP6_HUMAN antibody

  see all

Anti-MT-ATP6 antibody images

• 

  Western blot - Anti-MT-ATP6 antibody (ab102573)

  Anti-MT-ATP6 antibody (ab102573) at 1/250 dilution + Skin (Human) Tissue Lysate - adult normal tissue (ab30166) at 10 µg
  
  Secondary
  Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
  developed using the ECL technique
  
  Performed under reducing conditions.
  
  Predicted band size : 25 kDa
  Observed band size : 27 kDa (why is the actual band size different from the predicted?)
  Additional bands at : 46 kDa. We are unsure as to the identity of these extra bands.
  
  Exposure time : 20 minutes

• 

  Immunocytochemistry/ Immunofluorescence - Anti-MT-ATP6 antibody (ab102573)

  ICC/IF image of ab102573 stained HeLa cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody ab102573 at 5µg/ml overnight at +4°C. The secondary antibody (green) was DyLight® 488 goat anti- rabbit (ab96899) IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.

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