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ADCK3 Antibody

Datasheet
Code CSB-PA973086
Size US$297
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Image
  • Western blot analysis of extracts from HepG2 cells and mouse muscle cells, using ADCK3 antibody.
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) COQ8A Polyclonal antibody
Uniprot No.
Q8NI60
Target Names
COQ8A
Alternative Names
aarF domain containing protein kinase 3 antibody; aarF domain-containing protein kinase 3 antibody; ADCK 3 antibody; Adck3 antibody; ADCK3_HUMAN antibody; ARCA2 antibody; CABC 1 antibody; CABC1 antibody; Chaperone ABC1 (activity of bc1 complex S.pombe) like antibody; Chaperone ABC1 activity of bc1 complex homolog antibody; Chaperone ABC1 like antibody; Chaperone activity of bc1 complex like antibody; Chaperone activity of bc1 complex like mitochondrial antibody; Chaperone activity of bc1 complex-like antibody; Chaperone-ABC1-like antibody; Coenzyme Q8 homolog antibody; COQ 8 antibody; COQ10D4 antibody; COQ8 antibody; COQ8; S. cerevisiae; homolog of antibody; MGC4849 antibody; mitochondrial antibody; OTTHUMP00000035734 antibody; OTTHUMP00000035737 antibody; SCAR9 antibody
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Synthesized peptide derived from internal of Human ADCK3.
Immunogen Species
Homo sapiens (Human)
Clonality
Polyclonal
Purification Method
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration
It differs from different batches. Please contact us to confirm it.
Form
Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Tested Applications
ELISA,WB
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:3000
Protocols
ELISA Protocol
Western Blotting(WB) Protocol
Troubleshooting and FAQs
Antibody FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

Related Products

COQ8A Antibodies

COQ8A Proteins

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Target Background

Function
Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration. Its substrate specificity is unclear: does not show any protein kinase activity. 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. Shows an unusual selectivity for binding ADP over ATP.
Gene References into Functions
  1. Loss of COQ8a results in cerebellar ataxia and coenzyme Q deficiency. PMID: 27499294
  2. ADCK3/COQ8 localises to mitochondrial cristae and is targeted to this organelle via the presence of an N-terminal localisation signal PMID: 26866375
  3. work reveals Mg(2+)-dependent ATPase activity of ADCK3, providing strong support for the theoretical prediction of this protein being a functional atypical kinase. PMID: 25540914
  4. a structural foundation for investigating the role of transmembrane association in regulating the biological activity of ADCK3 PMID: 25216398
  5. Mitochondrial ADCK3 employs an atypical protein kinase-like fold to enable coenzyme Q biosynthesis. PMID: 25498144
  6. These observations highlight the importance of screening for a potentially treatable cause, CABC1/ADCK3 mutations PMID: 22036850
  7. play an important role in mediating p53-inducible apoptosis through the mitochondrial pathway. PMID: 11888884
  8. CABC1 gene mutations in four ubiquinone-deficient patients in three distinct families were reported. PMID: 18319072
  9. Five additional mutations in ADCK3 were found in three patients with sporadic ataxia, including one known to have CoQ10 deficiency in muscle. PMID: 18319074

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Involvement in disease
Coenzyme Q10 deficiency, primary, 4 (COQ10D4)
Subcellular Location
Mitochondrion. Membrane; Single-pass membrane protein.
Protein Families
Protein kinase superfamily, ADCK protein kinase family
Tissue Specificity
Widely expressed, with highest levels in adrenal gland, heart, pancreas, nasal mucosa, stomach, uterus and skeletal muscle.
Database Links

HGNC: 16812

OMIM: 606980

KEGG: hsa:56997

STRING: 9606.ENSP00000355739

UniGene: Hs.118241

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