COQ2,线粒体COQ2抗体

产品编号HZ-13984R
英文名称COQ2
中文名称线粒体COQ2抗体
别 名2310002F18Rik; 4-hydroxybenzoate polyprenyltransferase; CL640; Coenzyme Q2 homolog, prenyltransferase (yeast); COQ2; COQ2 homolog; COQ2_HUMAN; FLJ13014; FLJ26072; hCOQ2; MGC124824; MGC91278; mitochondrial; OTTMUSP00000032111; Para-hydroxybenzoate--polyprenyltransferase; Para-hydroxybenzoate--polyprenyltransferase, mitochondrial; PHB:polyprenyltransferase; RGD1306722.
说 明 书0.1ml 0.2ml
研究领域肿瘤 细胞生物 信号转导 细胞类型标志物 线粒体
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep,
COQ2,线粒体COQ2抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量37kDa
细胞定位细胞膜 线粒体
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human COQ2
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
COQ2,线粒体COQ2抗体PubMedPubMed
产品介绍background:
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. [provided by RefSeq, Oct 2009]

Function:
Catalyzes the prenylation of para-hydroxybenzoate (PHB) with an all-trans polyprenyl group. Mediates the second step in the final reaction sequence of coenzyme Q (CoQ) biosynthesis, which is the condensation of the polyisoprenoid side chain with PHB.

Subunit:

Subcellular Location:
Mitochondrion membrane.

Tissue Specificity:
Widely expressed. Present in all of the tissues tested. Expressed at higher level in skeletal muscle, adrenal glands and the heart.

Post-translational modifications:

DISEASE:
Defects in COQ2 are the cause of coenzyme Q10 deficiency, primary, type 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.

Similarity:
Belongs to the UbiA prenyltransferase family.

Gene ID:
27235

Database links:
Entrez Gene: 27235 Human
Omim: 609825 Human
SwissProt: Q96H96 Human
Unigene: 144304 Human
Unigene: 729069 Human

COQ2,线粒体COQ2抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.