COQ8A antibody (AA 501-647) (AbBy Fluor® 680)

Catalog No. ABIN4998873

Product Details anti-COQ8A Antibody (AbBy Fluor® 680)

Target: COQ8A (Coenzyme Q8A (COQ8A))

Binding Specificity: AA 501-647

Reactivity: Human, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This COQ8A antibody is conjugated to AbBy Fluor® 680

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

Cross-Reactivity: Human, Rat

Predicted Reactivity: Mouse,Dog,Cow,Sheep,Pig,Horse,Chicken,Rabbit

Purification: Purified by Protein A.

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

Isotype: IgG

Application Details

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

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

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

Preservative: ProClin

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

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for COQ8A

Target: COQ8A (Coenzyme Q8A (COQ8A))

Alternative Name: CABC1 (COQ8A Products)

Synonyms: ARCA2 antibody, CABC1 antibody, COQ10D4 antibody, COQ8 antibody, SCAR9 antibody, 4632432J16Rik antibody, AI462003 antibody, Cabc1 antibody, mKIAA0451 antibody, cabc1 antibody, Adck3 antibody, adck4 antibody, si:dkey-114g7.2 antibody, coenzyme Q8A antibody, aarF domain containing kinase 3 antibody, coenzyme Q8B antibody, COQ8A antibody, Coq8a antibody, coq8a antibody, ADCK3 antibody, coq8b antibody

Background:

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.