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Rabbit Anti-DHODH/BF594 Conjugated antibody (bs-14305R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-14305R-BF594
英文名稱 Rabbit Anti-DHODH/BF594 Conjugated antibody
中文名稱 BF594標(biāo)記的二氫乳清酸脫氫酶抗體
別    名 DHOdehase; Dhodh; Dihydroorotate dehydrogenase (quinone); Dihydroorotate dehydrogenase; Dihydroorotate dehydrogenase mitochondrial; Dihydroorotate oxidase; Human complement of yeast URA1; mitochondrial; PYRD_HUMAN.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng)
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 43kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DHODH
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:
The protein encoded by this gene catalyzes the fourth enzymatic step, the ubiquinone-mediated oxidation of dihydroorotate to orotate, in de novo pyrimidine biosynthesis. This protein is a mitochondrial protein located on the outer surface of the inner mitochondrial membrane. [provided by RefSeq, Jul 2008]

Function:
Catalyzes the conversion of dihydroorotate to orotate with quinone as electron acceptor.

Subcellular Location:
Mitochondrion inner membrane.

Post-translational modifications:
The uncleaved transit peptide is required for mitochondrial targeting and proper membrane integration.

DISEASE:
Defects in DHODH are the cause of postaxial acrofacial dysostosis (POADS) [MIM:263750]; also known as Miller syndrome. POADS is characterized by severe micrognathia, cleft lip and/or palate, hypoplasia or aplasia of the posterior elements of the limbs, coloboma of the eyelids and supernumerary nipples. POADS is a very rare disorder: only 2 multiplex families, each consisting of 2 affected siblings born to unaffected, nonconsanguineous parents, have been described among a total of around 30 reported cases.

Similarity:
Belongs to the dihydroorotate dehydrogenase family. Type 2 subfamily.

Database links:

Entrez Gene: 1723 Human

Entrez Gene: 533873 Cow

Entrez Gene: 610755 Dog

Entrez Gene: 56749 Mouse

Entrez Gene: 65156 Rat

Entrez Gene: 494065 Zebrafish

Omim: 126064 Human

SwissProt: Q02127 Human

SwissProt: O35435 Mouse

SwissProt: Q63707 Rat

Unigene: 654427 Human

Unigene: 23894 Mouse

Unigene: 81502 Rat



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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