COQ8A anticorps (AA 501-647) (AbBy Fluor® 594)

N° du produit ABIN2811006

Détail du produit anti-COQ8A anticorps (AbBy Fluor® 594)

Antigène: COQ8A (Coenzyme Q8A (COQ8A))

Épitope: AA 501-647

Reactivité: Humain, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp COQ8A est conjugé à/à la AbBy Fluor® 594

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

Réactivité croisée: Humain, Rat

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

Purification: Purified by Protein A.

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

Isotype: IgG

Information d'application

Indications d'application: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Stockage

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.

Agent conservateur: ProClin

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

Stock: -20 °C

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

Date de péremption: 12 months

Détails sur COQ8A

Antigène: COQ8A (Coenzyme Q8A (COQ8A))

Autre désignation: CABC1 (COQ8A Produits)

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

Sujet:

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.