Anti-MT-ATP6 antibody - C-terminal (ab190287)
Key features and details
- Goat polyclonal to MT-ATP6 - C-terminal
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
Overview
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Product name
Anti-MT-ATP6 antibody - C-terminal -
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 1-100 (C terminal) (Cysteine residue). The exact immunogen sequence used to generate this antibody is proprietary information. If additional detail on the immunogen is needed to determine the suitability of the antibody for your needs, please contact our Scientific Support team to discuss your requirements. YP_003024031.1
Database link: P00846 -
Positive control
- Human heart lysate
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General notes
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
Properties
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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...
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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
Associated products
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Compatible Secondaries
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Isotype control
Applications
The Abpromise guarantee
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 |
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WB |
Use a concentration of 0.1 - 0.3 µg/ml. Detects a band of approximately 26 kDa (predicted molecular weight: 25 kDa).
1 hour primary incubation is recommended for this product. |
Notes |
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WB
Use a concentration of 0.1 - 0.3 µg/ml. Detects a band of approximately 26 kDa (predicted molecular weight: 25 kDa). 1 hour primary incubation is recommended for this product. |
Target
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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
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Database links
- Entrez Gene: 4508 Human
- Omim: 516060 Human
- SwissProt: P00846 Human
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Alternative names
- ATP synthase subunit a antibody
- ATP6 antibody
- ATP6_HUMAN antibody
see all
Images
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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: 2 minutes
Datasheets and documents
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Datasheet download
References (0)
ab190287 has not yet been referenced specifically in any publications.