細胞色素氧化酶缺失蛋白2抗體 返回列表頁

細胞色素氧化酶缺失蛋白2抗體

英文名稱 SCO2
中文名稱 細胞色素氧化酶缺失蛋白2抗體
別    名 Cytochrome oxidase deficient homolog 2; MGC125823; MGC125825; OTTHUMP00000196774; OTTHUMP00000196775; Protein SCO2 homolog, mitochondrial; SCO (cytochrome oxidase deficient, yeast) homolog 2; SCO 1L; SCO 2; SCO cytochrome oxidase deficient homolog 2 (yeast); SCO cytochrome oxidase deficient homolog 2; SCO1L; SCO2; SCO2_HUMAN; Synthesis of cytochrome c oxidase 2.
  
說 明 書 0.2ml  
研究領域 腫瘤  細胞生物  信號轉(zhuǎn)導  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat, 
產(chǎn)品應用 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 （石蠟切片需做抗原修復） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 25kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human SCO2
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.

PubMed PubMed
產(chǎn)品介紹 background:
Cytochrome c oxidase (COX) catalyzes the transfer of electrons from cytochrome c to molecular oxygen, which helps to maintain the proton gradient across the inner mitochondrial membrane that is necessary for aerobic ATP production. Human COX is a multimeric protein complex that requires several assembly factors; this gene encodes one of the COX asembly factors. The encoded protein is a metallochaperone that is involved in the biogenesis of cytochrome c oxidase subunit II. Mutations in this gene are associated with fatal infantile encephalocardiomyopathy. [provided by RefSeq, Dec 2009]

Function:
Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2).

Subcellular Location:
Mitochondrion.

Tissue Specificity:
Ubiquitous.

DISEASE:
Defects in SCO2 are the cause of fatal infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC) [MIM:604377]. This disease is characterized by hypertrophic cardiomyopathy, lactic acidosis, and gliosis. Heart and skeletal muscle show reductions in cytochrome c oxidase (COX) activity, whereas liver and fibroblasts show mild COX deficiencies.

Similarity:
Belongs to the SCO1/2 family.
Contains 1 thioredoxin domain.xy-15021R    C1orf1411號染色體開放閱讀框141抗體
xy-15022R    C1orf1441號染色體開放閱讀框144抗體
xy-12464R    alpha COP ICOPA蛋白抗體
xy-15045R    C1orf1851號染色體開放閱讀框185抗體
xy-15969R    phospho-alpha B Crystallin (Ser19)磷酸化熱休克蛋白β5/α晶狀體球蛋白B/αB-crystallin抗體
xy-12970R    phospho-alpha B Crystallin (Ser45)磷酸化α晶狀體球蛋白B/αB-crystallin抗體
xy-12971R    phospho-alpha B Crystallin (Ser53)磷酸化熱休克蛋白β5/α晶狀體球蛋白B/αB-crystallin抗體
xy-15034R    C1orf1671號染色體開放閱讀框167抗體
xy-15035R    C1orf168 1號染色體開放閱讀框168抗體
xy-15036R    C1orf1701號染色體開放閱讀框170抗體
xy-15037R    C1orf1721號染色體開放閱讀框172抗體
xy-15038R    C1orf173 1號染色體開放閱讀框173抗體
xy-15039R    C1orf1741號染色體開放閱讀框174抗體
xy-15040R    C1orf1771號染色體開放閱讀框177抗體
xy-15041R    C1orf180 1號染色體開放閱讀框180抗體
xy-15043R    C1orf182 1號染色體開放閱讀框182抗體
xy-15044R    C1orf1831號染色體開放閱讀框183抗體
xy-15047R    C1orf1871號染色體開放閱讀框187抗體
xy-15048R    C1orf189 1號染色體開放閱讀框189抗體
xy-15049R    C1ORF1901號染色體開放閱讀框190抗體
xy-15051R    C1orf1921號染色體開放閱讀框192抗體
xy-15052R    C1orf1941號染色體開放閱讀框194抗體
xy-15054R    C1orf1981號染色體開放閱讀框198抗體
xy-15057R    C1orf211號染色體開放閱讀框21抗體
xy-15058R    C1orf2161號染色體開放閱讀框216抗體
xy-15066R    C1orf43 1號染色體開放閱讀框43抗體
xy-15068R    C1orf501號染色體開放閱讀框50抗體
xy-15067R    C1orf491號染色體開放閱讀框49抗體
xy-15069R    C1orf531號染色體開放閱讀框53抗體
xy-15070R    C1orf541號染色體開放閱讀框54抗體
xy-15071R    C1orf551號染色體開放閱讀框55抗體
xy-15072R    C1orf561號染色體開放閱讀框56抗體