COQ8A Antikörper (AA 501-647) (AbBy Fluor® 647)

Produktnummer ABIN881438

Der Kaninchen Polyklonal Anti-COQ8A-Antikörper wurde für WB, IF (cc) und IF (p) validiert. Er ist geeignet, COQ8A in Proben von Human und Ratte zu detektieren.

Kurzübersicht für COQ8A Antikörper (AA 501-647) (AbBy Fluor® 647) (ABIN881438)

Target: COQ8A (Coenzyme Q8A (COQ8A))

Reaktivität: Human, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser COQ8A Antikörper ist konjugiert mit AbBy Fluor® 647

Applikation: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Produktdetails anti-COQ8A Antikörper (AbBy Fluor® 647)

Bindungsspezifität: AA 501-647

Kreuzreaktivität: Human, Ratte

Homologie: Mouse,Dog,Cow,Sheep,Pig,Horse,Chicken,Rabbit

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human ADCK3/CABC1

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Konservierungsmittel: ProClin

Vorsichtsmaßnahmen: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu COQ8A

Target: COQ8A (Coenzyme Q8A (COQ8A))

Andere Bezeichnung: CABC1

Hintergrund:

Synonyms: mitochondrial, aarF domain containing protein kinase 3, aarF domain-containing protein kinase 3, ADCK 3, ADCK3, ADCK3_HUMAN, CABC 1, Chaperone ABC1 activity of bc1 complex S.pombe like, Chaperone ABC1 activity of bc1 complex homolog, Chaperone ABC1 like, Chaperone activity of bc1 complex like, Chaperone activity of bc1 complex like mitochondrial, Chaperone activity of bc1 complex-like, Chaperone-ABC1-like, Coenzyme Q8 homolog, COQ 8, COQ8.

Background: May be a chaperone-like protein essential for the proper conformation and functioning of protein complexes in the respiratory chain.Tissue specificity:Ubiquitously expressed with a relatively greater abundance in heart and skeletal muscle.Involvement in disease:Defects in ADCK3 are a cause of coenzyme Q10 deficiency (CoQ10 deficiency). CoQ10 deficiency is an autosomal recessive disorder with variable manifestations. It can be associated with three main clinical phenotypes: a predominantly myopathic form with central nervous system involvement, an infantile encephalomyopathy with renal dysfunction and an ataxic form with cerebellar atrophy.Defects in ADCK3 are the cause of spinocerebellar ataxia autosomal recessive type 9 (SCAR9) [MIM:612016], also known as autosomal recessive cerebellar ataxia type 2 (ARCA2). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCAR9 is an autosomal recessive form characterized by gait ataxia and cerebellar atrophy with slow progression and few associated features. Patients can manifest brisk tendon reflexes and Hoffmann sign, mild psychomotor retardation, mild axonal degeneration of the sural nerve, exercise intolerance and elevated serum lactate.