Anti-COQ8A Antibody

$99.00$319.00

Reactivity: Human
Applications: ELISA,IHC
Conjugation: Various
Supplier: CUSABIO BIOTECH CO.

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Rabbit Anti-COQ8A Antibody (CSB-PA850918ESR1HU)

Supplier: CUSABIO BIOTECH CO.

Alternative names:

Chaperone activity of bc1 complex-like Antibody,Chaperone-ABC1-like Antibody,aarF domain-containing protein kinase 3 Antibody,ADCK3 Antibody,CABC1 Antibody

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Recommended applications: ELISA, IHC

Recommended dilution: Recommended dilution:IHC:1:20-1:200

Recommended protocols: check protocols

Name

Anti-COQ8A Antibody

Catalogue No.

CSB-PA850918ESR1HU

Reactivity

Human

Immunogen

Recombinant human Atypical kinase ADCK3, mitochondrial protein (1-220AA)

Host

Rabbit

Applications

ELISA, IHC

Recommended dilution

Recommended dilution:IHC:1:20-1:200

Clonality

Polyclonal

Conjugation

unconjugated

Isotype

IgG

Molecular weight

71 kDa

Purification

Antigen Affinity Purified

Storage

Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.

Protein function

Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration (PubMed:25498144, PubMed:21296186, PubMed:25540914, PubMed:27499294). Its substrate specificity is unclear: does not show any protein kinase activity (PubMed:25498144, PubMed:27499294). Probably acts as a small molecule kinase, possibly a lipid kinase that phosphorylates a prenyl lipid in the ubiquinone biosynthesis pathway, as suggested by its ability to bind coenzyme Q lipid intermediates (PubMed:25498144, PubMed:27499294). Shows an unusual selectivity for binding ADP over ATP (PubMed:25498144). .

Protein tissue specificity

Widely expressed, with highest levels in adrenal gland, heart, pancreas, nasal mucosa, stomach, uterus and skeletal muscle. .

Involvement in disease

Coenzyme Q10 deficiency, primary, 4 (COQ10D4) [MIM:612016]: An autosomal recessive disorder characterized by childhood-onset of cerebellar ataxia and exercise intolerance. Patient manifest gait ataxia and cerebellar atrophy with slow progression. Additional features include brisk tendon reflexes and Hoffmann sign, variable psychomotor retardation and variable seizures. . Note=The disease is caused by mutations affecting the gene represented in this entry.

Protein sequence and domain

Belongs to the protein kinase superfamily. ADCK protein kinase family. Adopts an atypical protein kinase-like fold: while it adopts a core fold similar to that of well-characterized protein kinase-like domains, a number of features are positioned to inhibit the kinase activity: (1) an atypical AAAS motif in an alanine-rich (A-rich) loop that replaces the canonical glycine-rich (G-rich) nucleotide-binding loop and limits ATP binding by establishing an unusual selectivity for ADP and (2) an N-terminal domain, containing the KxGQ motif, that completely occludes the typical substrate binding pocket (PubMed:25498144). Nucleotide-binding opens the substrate binding pocket and flips the active site from inside the hydrophobic core into a catalytically competent, solvent-exposed posture (PubMed:27499294).

Protein cellular localization

Mitochondrion . Membrane ; Single-pass membrane protein .

Note

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Supplier

CUSABIO BIOTECH CO.

Product type

Primary antibody

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