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COQ8A encodes a mitochondrial protein similar to yeast ABC1, which functions in an electron-transferring membrane protein complex in the respiratory chain. Additionally we are shipping Coenzyme Q8A Proteins (5) and and many more products for this protein.
Showing 10 out of 107 products:
Human Monoclonal COQ8A Primary Antibody for ELISA, WB - ABIN528051
OConnell, Guo, Serra, Beck, Wachtman, Hoggatt, Xia, Pearson, Knight, OConnell, Miller, Westmoreland, Bhasin: The effects of an ActRIIb receptor Fc fusion protein ligand trap in juvenile simian immunodeficiency virus-infected rhesus macaques. in FASEB journal : official publication of the Federation of American Societies for Experimental Biology 2015
Show all 2 Pubmed References
Cow (Bovine) Polyclonal COQ8A Primary Antibody for WB - ABIN2783345
Lagier-Tourenne, Tazir, López, Quinzii, Assoum, Drouot, Busso, Makri, Ali-Pacha, Benhassine, Anheim, Lynch, Thibault, Plewniak, Bianchetti, Tranchant, Poch, DiMauro, Mandel, Barros, Hirano, Koenig: ADCK3, an ancestral kinase, is mutated in a form of recessive ataxia associated with coenzyme Q10 deficiency. in American journal of human genetics 2008
Loss of COQ8a results in cerebellar ataxia and coenzyme Q deficiency.
ADCK3/COQ8 localises to mitochondrial cristae and is targeted to this organelle via the presence of an N-terminal localisation signal
work reveals Mg(2 (show MUC7 Antibodies)+)-dependent ATPase (show DNAH8 Antibodies) activity of ADCK3, providing strong support for the theoretical prediction of this protein being a functional atypical kinase.
a structural foundation for investigating the role of transmembrane association in regulating the biological activity of ADCK3
Mitochondrial ADCK3 employs an atypical protein kinase-like fold to enable coenzyme Q biosynthesis.
These observations highlight the importance of screening for a potentially treatable cause, CABC1/ADCK3 mutations
play an important role in mediating p53 (show TP53 Antibodies)-inducible apoptosis through the mitochondrial pathway.
CABC1 gene mutations in four ubiquinone-deficient patients in three distinct families were reported.
Five additional mutations in ADCK3 were found in three patients with sporadic ataxia, including one known to have CoQ10 deficiency in muscle.
This gene encodes a mitochondrial protein similar to yeast ABC1, which functions in an electron-transferring membrane protein complex in the respiratory chain. It is not related to the family of ABC transporter proteins. Expression of this gene is induced by the tumor suppressor p53 and in response to DNA damage, and inhibiting its expression partially suppresses p53-induced apoptosis. Alternatively spliced transcript variants have been found\; however, their full-length nature has not been determined.
aarF domain-containing protein kinase 3
, chaperone activity of bc1 complex-like, mitochondrial
, chaperone, ABC1 activity of bc1 complex homolog
, coenzyme Q8 homolog
, chaperone, ABC1 activity of bc1 complex homolog (S. pombe)
, chaperone, ABC1 activity of bc1 complex like
, chaperone, ABC1 activity of bc1 complex-like
, aarF domain containing kinase 3
, chaperone activity of bc1 complex-like, mitochondrial-like
, atypical kinase ADCK3, mitochondrial
, AarF domain-containing protein kinase 4
, Coenzyme Q protein 8B
, aarF domain containing kinase 4
, aarF domain-containing protein kinase 4
, atypical kinase COQ8B, mitochondrial
, coenzyme Q protein 8B