Información del producto
Información adicional
Storage Conditions
-20°C/1 year
Applications
ELISA
IHC
IHC
Concentration
1 mg/ml
Recomended Dilution
IHC-p 1:50-200, ELISA(peptide)1:5000-20000
Type Clonality
Polyclonal
Immunogen
Synthesized peptide derived from human MT-ATP8 AA range: 30-110
Subcellular Location
Mitochondrion membrane
Single-pass membrane protein.
Single-pass membrane protein.
Iso type
IgG
Tissue Source
Rabbit
Reactivity
Human
Mouse
Rat
Mouse
Rat
Human Gen
4509
Other Name
ATP synthase protein 8 (A6L
F-ATPase subunit 8)
F-ATPase subunit 8)
Background
also called mitochondrial complex IV deficiency. COX deficiency is a clinically heterogeneous disorder. The clinical features are ranging from isolated myopathy to severe multisystem disease, with onset from infancy to adulthood.,disease:Defects in MT-CO3
disease:Defects in MT-ATP6 are a cause of infantile bilateral striatal necrosis [MIM:500003]. Bilateral striatal necrosis is a neurological disorder resembling Leigh syndrome.,disease:Defects in MT-ATP6 are a cause of Leber hereditary optic neuropathy (LH
it may play a direct role in the translocation of protons across the membrane.,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 genera
disease:Defects in MT-ATP6 are a cause of infantile bilateral striatal necrosis [MIM:500003]. Bilateral striatal necrosis is a neurological disorder resembling Leigh syndrome.,disease:Defects in MT-ATP6 are a cause of Leber hereditary optic neuropathy (LH
it may play a direct role in the translocation of protons across the membrane.,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 genera