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Rabbit Anti-CYP4V2/BF488 Conjugated antibody (bs-14161R-BF488)
~~~促销,代码KXJ230206-6~~~
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-14161R-BF488
英文名称 Anti-CYP4V2/BF488
中文名称 BF488标记的细胞色素P450 4V2抗体
别    名 BCD; CP4V2_HUMAN; CYP4AH1; CYP4V 2; CYP4V2; Cytochrome P450 4V2; Cytochrome P450, family 4, subfamily V, polypeptide 2; Retina CYP4V2.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  免疫学  信号转导  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, )
产品应用 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 61kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CYP4V2
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存条件 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.
产品介绍 background:
This gene encodes a member of the cytochrome P450 hemethiolate protein superfamily which are involved in oxidizing various substrates in the metabolic pathway. It is implicated in the metabolism of fatty acid precursors into n-3 polyunsaturated fatty acids. Mutations in this gene result in Bietti crystalline corneoretinal dystrophy. [provided by RefSeq, Jul 2008]

Function:
Catalyzes the omega-hydroxylation of medium-chain saturated fatty acids such as laurate, myristate and palmitate in an NADPH-dependent pathway. The substrate specificity is higher for myristate > laurate > palmitate (C14>C16>C12). May have a role in fatty acid and steroid metabolism in the eye.

Subcellular Location:
Endoplasmic reticulum membrane.

Tissue Specificity:
Broadly expressed. Detected in heart, brain, placenta, lung, liver, skeletal muscle, kidney, pancreas, retina, retinal pigment epithelium (RPE) and lymphocytes.

DISEASE:
Bietti crystalline corneoretinal dystrophy (BCD) [MIM:210370]: An autosomal recessive ocular disease characterized by retinal degeneration and marginal corneal dystrophy. Typical features include multiple glistening intraretinal crystals scattered over the fundus, a characteristic degeneration of the retina, and sclerosis of the choroidal vessels, ultimately resulting in progressive night blindness and constriction of the visual field. Most patients have similar crystals at the corneoscleral limbus. Patients develop decreased vision, nyctalopia, and paracentral scotomata between the 2nd and 4th decade of life. Later, they develop peripheral visual field loss and marked visual impairment, usually progressing to legal blindness by the 5th or 6th decade of life. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the cytochrome P450 family.

Database links:

Entrez Gene: 285440 Human

Entrez Gene: 102294 Mouse

Entrez Gene: 266761 Rat

Omim: 608614 Human

SwissProt: Q6ZWL3 Human

SwissProt: Q9DBW0 Mouse

SwissProt: A2RRT9 Rat

Unigene: 587231 Human

Unigene: 245297 Mouse

Unigene: 201722 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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