COQ6 Antibody, FITC conjugated (PACO51260)
- SKU:
- PACO51260
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- FITC
Frequently bought together:
Description
抗体名: | COQ6 Antibody, FITC conjugated (PACO51260) |
抗体コード: | PACO51260 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial protein (208-339AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | FITC |
バックグラウンド: | FAD-dependent monooxygenase required for the C5-ring hydroxylation during ubiquinone biosynthesis. Catalyzes the hydroxylation of 3-polyprenyl-4-hydroxybenzoic acid, to 3-polyprenyl-4,5-dihydroxybenzoic acid, The electrons required for the hydroxylation reaction may be funneled indirectly from NADPH via a ferredoxin/ferredoxin reductase system to COQ6. |
シノニム: | Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial (EC 1.14.13) (Coenzyme Q10 monooxygenase 6), COQ6 |
UniProt Protein Function: | COQ6: Defects in COQ6 are the cause of coenzyme Q10 deficiency, primary, type 6 (COQ10D6). An autosomal recessive disorder characterized by onset in infancy of severe progressive nephrotic syndrome resulting in end-stage renal failure and sensorineural deafness. Renal biopsy usually shows focal segmental glomerulosclerosis. Belongs to the UbiH/COQ6 family. 3 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Cofactor and Vitamin Metabolism - ubiquinone and other terpenoid-quinone biosynthesis; EC 1.14.13.-; Mitochondrial; Oxidoreductase Chromosomal Location of Human Ortholog: 14q24.3 Cellular Component: mitochondrion Disease: Coenzyme Q10 Deficiency, Primary, 6 |
NCBI Summary: | The protein encoded by this gene belongs to the ubiH/COQ6 family. It is an evolutionarily conserved monooxygenase required for the biosynthesis of coenzyme Q10 (or ubiquinone), which is an essential component of the mitochondrial electron transport chain, and one of the most potent lipophilic antioxidants implicated in the protection of cell damage by reactive oxygen species. Knockdown of this gene in mouse and zebrafish results in decreased growth due to increased apoptosis. Mutations in this gene are associated with autosomal recessive coenzyme Q10 deficiency-6 (COQ10D6), which manifests as nephrotic syndrome with sensorineural deafness. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Jun 2012] |
UniProt Code: | Q9Y2Z9 |
NCBI GenInfo Identifier: | 26006952 |
NCBI Gene ID: | 51004 |
NCBI Accession: | Q9Y2Z9.2 |
UniProt Secondary Accession: | Q9Y2Z9,Q53GG6, Q86U30, Q96CA1, Q96CK2, B7Z3K8, |
UniProt Related Accession: | Q9Y2Z9 |
Molecular Weight: | 51kDa |
NCBI Full Name: | Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial |
NCBI Synonym Full Names: | coenzyme Q6, monooxygenase |
NCBI Official Symbol: | COQ6 |
NCBI Official Synonym Symbols: | CGI10; CGI-10; COQ10D6 |
NCBI Protein Information: | ubiquinone biosynthesis monooxygenase COQ6, mitochondrial |
UniProt Protein Name: | Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial |
UniProt Synonym Protein Names: | Coenzyme Q10 monooxygenase 6 |
UniProt Gene Name: | COQ6 |