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Rabbit Anti-COQ2/Gold Conjugated antibody (bs-13984R-Gold)
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說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產品編號 bs-13984R-Gold
英文名稱1 Rabbit Anti-COQ2/Gold Conjugated antibody
中文名稱 膠體金標記的線粒體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)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領域 腫瘤  細胞生物  信號轉導  細胞類型標志物  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep, )
產品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
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 0.4mg/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 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產品介紹 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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