Anti-COQ2 monoclonal antibody (DCABH-11095) Made to order

Rabbit anti-Human COQ2 monoclonal antibody for WB, ELISA

View other COQ2 antibodies

Specifications


Host Species
Rabbit
Antibody Isotype
IgG
Species Reactivity
Human
Immunogen
A synthetic peptide of human COQ2 is used for rabbit immunization.
Conjugate
Unconjugated

Target


Alternative Names
COQ2; coenzyme Q2 homolog, prenyltransferase (yeast); 4-hydroxybenzoate polyprenyltransferase, mitochondrial; 4 hydroxybenzoate polyprenyltransferase; CL640; FLJ26072
Entrez Gene ID
UniProt ID

Product Background


Gene summary
COQ2 (Coenzyme Q2, Polyprenyltransferase) is a Protein Coding gene. Diseases associated with COQ2 include coenzyme q10 deficiency, primary, 1 and multiple system atrophy. Among its related pathways are Metabolism and Transport to the Golgi and subsequent modification. GO annotations related to this gene include prenyltransferase activity and 4-hydroxybenzoate nonaprenyltransferase activity. This gene encodes an enzyme that functions in the final steps in the biosynthesis of CoQ (ubiquinone), a redox carrier in the mitochondrial respiratory chain and a lipid-soluble antioxidant. This enzyme, which is part of the coenzyme Q10 pathway, catalyzes the prenylation of parahydroxybenzoate with an all-trans polyprenyl group. Mutations in this gene cause coenzyme Q10 deficiency, a mitochondrial encephalomyopathy, and also COQ2 nephropathy, an inherited form of mitochondriopathy with primary renal involvement.
Antigen Description
CoQ (ubiquinone) serves as a redox carrier in the mitochondrial respiratory chain and is a lipid-soluble antioxidant. COQ2, or parahydroxybenzoate-polyprenyltransferase (EC 2. 5. 1. 39), catalyzes one of the final reactions in the biosynthesis of CoQ, the prenylation of parahydroxybenzoate with an all-trans polyprenyl group (Forsgren et al. , 2004 [PubMed 15153069]). Coenzyme Q10 deficiency, primary, 1 (COQ10D1) [MIM:607426]: An autosomal recessive disorder with variable manifestations consistent with 5 major phenotypes. The phenotypes include an encephalomyopathic form with seizures and ataxia; a multisystem infantile form with encephalopathy, cardiomyopathy and renal failure; a predominantly cerebellar form with ataxia and cerebellar atrophy; Leigh syndrome with growth retardation; and an isolated myopathic form. Note=The disease is caused by mutations affecting the gene represented in this entry. Multiple system atrophy 1 (MSA1) [MIM:146500]: A progressive neurodegenerative disorder clinically characterized by parkinsonism, cerebellar ataxia, and autonomic, urogenital, and pyramidal dysfunction in various combinations. Pathologically, it is characterized by degeneration of striatonigral and olivopontocerebellar structures, and glial cytoplasmic inclusions that consist of abnormally phosphorylated alpha-synuclein or tau. Note=Disease susceptibility is associated with variations affecting the gene represented in this entry. Para-hydroxybenzoate—polyprenyltransferase, mitochondrial is an enzyme that in humans is encoded by the COQ2 gene. 0The function about COQ2 antigen include 4-hydroxybenzoate decaprenyltransferase activity; 4-hydroxybenzoate nonaprenyltransferase activity; prenyltransferase activity; transferase activity.
Pathway
Metabolic pathways, organism-specific biosystem; Metabolism of proteins, organism-specific biosystem; Mitochondrial Protein Import, organism-specific biosystem; Ubiquinone and other terpenoid-quinone biosynthesis, organism-specific biosystem; Ubiquinone and other terpenoid-quinone biosynthesis, conserved biosystem; ubiquinol-1 biosynthesis (eukaryotic), conserved biosystem.

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References


Cheng, W; Song, C; et al. Coenzyme Q plays opposing roles on bacteria/fungi and viruses in Drosophila innate immunity. INTERNATIONAL JOURNAL OF IMMUNOGENETICS 38:331-337(2011).
Buron, MI; Herman, MD; et al. Stimulation of polyprenyl 4-hydroxybenzoate transferase activity by sodium cholate and 3-[(cholamidopropyl) dimethylammonio]-1-propanesulfonate. ANALYTICAL BIOCHEMISTRY 353:15-21(2006).

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