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天干天干天啪啪夜爽爽99|99精品国产一区二区
Rabbit Anti-COQ2/BF488 Conjugated antibody (bs-13984R-BF488)
訂購(gòu)熱線(xiàn):400-901-9800
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-13984R-BF488
英文名稱(chēng)1 Rabbit Anti-COQ2/BF488 Conjugated antibody
中文名稱(chēng) BF488標(biāo)記的線(xiàn)粒體COQ2抗體
別    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.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞類(lèi)型標(biāo)志物  線(xiàn)粒體  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 37kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human COQ2
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 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.
產(chǎn)品介紹 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.

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.

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.

Database links:

Entrez Gene: 27235 Human

Omim: 609825 Human

SwissProt: Q96H96 Human

Unigene: 144304 Human

Unigene: 729069 Human



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