mm1313亚洲精品,欧美俄罗斯40老熟妇,欧美日韩在线观看视频在线,亚洲欧美国产激情综合在线

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  人才招聘  關于我們  聯(lián)系我們
亚洲欧洲视频在线观看网站,欧美日韩中文一区字幕,91免费精品国偷自产在线在线
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-COQ2/RBITC Conjugated antibody (bs-13984R-RBITC)
訂購熱線:400-901-9800
訂購郵箱:sales@www.p2b3.cn
訂購QQ:  400-901-9800
技術支持:techsupport@www.p2b3.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-13984R-RBITC
英文名稱1 Rabbit Anti-COQ2/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)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  
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 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 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.
版權所有 2004-2026 www.www.p2b3.cn 北京博奧森生物技術有限公司
通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫(yī)療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網安備110107000727號
岗巴县| 焦作市| 布尔津县| 内黄县| 漳浦县| 连平县| 西安市| 高邮市| 霍邱县| 郯城县| 池州市| 泰顺县| 淅川县| 洪雅县| 襄垣县| 正宁县| 密山市| 江陵县| 长兴县| 石台县| 黄大仙区| 邢台市| 康平县| 海门市| 常宁市| 洛南县| 元阳县| 新竹县| 黎城县| 沂源县| 集安市| 布尔津县| 阳春市| 马山县| 三穗县| 弥勒县| 区。| 南江县| 施秉县| 西和县| 永春县|