ATP6 rabbit pAb
Produktgrößen
50UL
£ POA
ES9414-50UL
100UL
£ POA
ES9414-100UL
Über dieses Produkt
- SKU:
- ES9414
- Anwendung:
- Western Blot, ELISA
- Klonalität:
- Polyclonal
- Konzentration:
- 1 mg/ml
- Weitere Details:
- 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 (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.;disease: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.;disease:Defects in MT-ATP6 are the cause of neurogenic muscle weakness; ataxia; and retinitis pigmentosa (NARP) [MIM:551500].;disease:Defects in MT-CO3 are a cause of cytochrome c oxidase deficiency (COX deficiency) [MIM:220110]; 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 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.;disease:Defects in MT-CO3 are associated with recurrent myoglobinuria [MIM:550500]. Myoglobinuria consists of excretion of myoglobin in the urine.;disease:Defects in MT-CO3 are found in mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome; a genetically heterogeneous disorder; characterized by episodic vomiting; seizures; and recurrent cerebral insults resembling strokes and causing hemiparesis; hemianopsia; or cortical blindness.;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.;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. Part of the complex F(0) domain. Minor subunit located with subunit a in the membrane.;function:Subunits I; II and III form the functional core of the enzyme complex.;similarity:Belongs to the ATPase A chain family.;similarity:Belongs to the ATPase protein 8 family.;similarity:Belongs to the cytochrome c oxidase subunit 3 family.;subunit:F-type ATPases have 2 components; CF(1) - the catalytic core - and CF(0) - the membrane proton channel.;subunit:F-type ATPases have 2 components; CF(1) - the catalytic core - and CF(0) - the membrane proton channel. CF(1) has five subunits: alpha(3); beta(3); gamma(1); delta(1); epsilon(1). CF(0) has three main subunits: a; b and c.;
- Immunogen:
- Synthesized peptide derived from human protein . at AA range: 60-140
- Isotyp:
- IgG
- Reaktivitäten:
- Human, Rat, Mouse
- Versandbedingungen:
- Blue Ice
- Quelle:
- Rabbit
- Lagerbedingungen:
- -20[o]C
- Hersteller:
- ELK Biotechnology
- Typ:
- Antibodies: Polyclonal Antibody
