Immunofluorescence (IF)
(Immunofluorescence analysis of L929 cells using MT-ATP6 Polyclonal Antibody at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.)
Rabbit MT-ATP6 Polyclonal Antibody | anti-MT-ATP6 antibody
MT-ATP6 Polyclonal Antibody
Immunofluorescence (IF)
(Immunofluorescence analysis of L929 cells using MT-ATP6 Polyclonal Antibody at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.)
Immunofluorescence (IF)
(Immunofluorescence analysis of L929 cells using MT-ATP6 Polyclonal Antibody at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.)
NCBI and Uniprot Product Information
Uniprot Description
ATP6: 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. Defects in MT-ATP6 are the cause of neurogenic muscle weakness, ataxia, and retinitis pigmentosa (NARP). Defects in MT-ATP6 are a cause of Leber hereditary optic neuropathy (LHON). 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). 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). Bilateral striatal necrosis is a neurological disorder resembling Leigh syndrome. Belongs to the ATPase A chain family.
Protein type: Transporter, ion channel; Membrane protein, multi-pass; Mitochondrial; Membrane protein, integral; Transporter
Chromosomal Location of Human Ortholog: -
Disease: Neuropathy, Ataxia, And Retinitis Pigmentosa; Leber Optic Atrophy
Research Articles on MT-ATP6
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Product Notes
The MT-ATP6 mt-atp6 (Catalog #AAA2566100) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The MT-ATP6 Polyclonal Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's MT-ATP6 can be used in a range of immunoassay formats including, but not limited to, Immunofluorescence (IF). IF: 1:50-1:100. Researchers should empirically determine the suitability of the MT-ATP6 mt-atp6 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "MT-ATP6, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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