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Anti-MT-ATP6 Antibody (CAB17960)

SKU:
CAB17960
Product Type:
Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology
€139 - €419
Frequently bought together:

Description

system_update_altDatasheetsystem_update_altMSDS
Antibody Name:Anti-MT-ATP6 Antibody
Antibody SKU:CAB17960
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC IF
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant protein of human MT-ATP6.
Application:WB IHC IF
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:100 IF 1:50 - 1:100
Reactivity:Human, Mouse, Rat
Positive Samples:HeLa, U-251MG, mouse brain, mouse heart, mouse kidney, rat kidney
Immunogen:Recombinant protein of human MT-ATP6.
Purification Method:Affinity purification
Storage Buffer:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:4508
Uniprot:P00846
Cellular Location:
Calculated MW:25kDa
Observed MW:25kDa
Anti-MT-ATP6 Antibody (CAB17960)Western blot - MT-ATP6 Rabbit pAb (CAB17960)
Synonyms:ATPase6, MTATP6, MT-ATP6
Background:
UniProt Protein Function: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.
UniProt Protein Details:

Protein type:Transporter, ion channel; Membrane protein, integral; Mitochondrial; Membrane protein, multi-pass; Transporter

Chromosomal Location of Human Ortholog: -

Disease: Neuropathy, Ataxia, And Retinitis Pigmentosa; Leber Optic Atrophy

UniProt Code:P00846
NCBI GenInfo Identifier:251831112
NCBI Gene ID:4508
NCBI Accession:YP_003024031.1
UniProt Secondary Accession:P00846,Q34772, Q5S8W5, Q5S9E7, Q5S9I6, Q5SA31, Q6RPB7 Q6VHC0, Q6VHE0, Q6WQF4, Q7YCC1, Q7YCF8,
Molecular Weight:25kDa
NCBI Full Name:ATP synthase F0 subunit 6 (mitochondrion)
NCBI Synonym Full Names:mitochondrially encoded ATP synthase 6
NCBI Official Symbol:MT-ATP6  
NCBI Official Synonym Symbols:ATPase6; MTATP6; ATP6  
NCBI Protein Information:ATP synthase F0 subunit 6
UniProt Protein Name:ATP synthase subunit a
UniProt Synonym Protein Names:F-ATPase protein 6
UniProt Gene Name:MT-ATP6  
UniProt Entry Name:ATP6_HUMAN

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