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货号： bs-14572R-Cy3 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-14572R-Cy3
- 英文名称
- Anti-ELOVL4/Cy3
- 中文名称
- Cy3标记的长链脂肪酸延长酶ELOVL4抗体
- 别 名
- 3-keto acyl-CoA synthase ELOVL4; ADMD; Cancer/testis antigen 118; CT118; Elongation of very long chain fatty acids (FEN1/Elo2 SUR4/Elo3 yeast) like 4; elongation of very long chain fatty acids (FEN1/Elo2 SUR4/Elo3 yeast)-like 4; Elongation of very long chain fatty acids like 4; Elongation of very long chain fatty acids protein 4; ELOV L4; ELOV4_HUMAN; ELOVL 4; ELOVL4; FLJ17667; FLJ92876; Stargardt disease 3; Stargardt disease 3 autosomal dominant; STGD 2; STGD 3; STGD2; STGD3.
- 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 神经生物学 转录调节因子 新陈代谢
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Horse, Rabbit,
- 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
- 37kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human ELOVL4
- 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 membrane-bound protein which is a member of the ELO family, proteins which participate in the biosynthesis of fatty acids. Consistent with the expression of the encoded protein in photoreceptor cells of the retina, mutations and small deletions in this gene are associated with Stargardt-like macular dystrophy (STGD3) and autosomal dominant Stargardt-like macular dystrophy (ADMD), also referred to as autosomal dominant atrophic macular degeneration. [provided by RefSeq, Jul 2008]
Function:
Condensing enzyme that elongates saturated and monounsaturated very long chain fatty acids (VLCFAs). Elongates C24:0 and C26:0 acyl-CoAs. Seems to represent a photoreceptor-specific component of the fatty acid elongation system residing on the endoplasmic reticulum. May be implicated in docosahexaenoic acid (DHA) biosynthesis, which requires dietary consumption of the essential alpha-linolenic acid and a subsequent series of three elongation steps
Subunit:
Oligomer.
Subcellular Location:
Endoplasmic reticulum membrane.
Tissue Specificity:
Expressed in the retina and at much lower level in the brain. Ubiquitous, highest expression in thymus, followed by testis, small intestine, ovary, and prostate. Little or no espression in heart, lung, liver, or leukocates.
DISEASE:
Defects in ELOVL4 are the cause of Stargardt disease type 3 (STGD3) [MIM:600110]. STGD is one of the most frequent causes of macular degeneration in childhood. It is characterized by macular dystrophy with juvenile-onset, rapidly progressive course, alterations of the peripheral retina, and subretinal deposition of lipofuscin-like material. STGD3 inheritance is autosomal dominant.
Similarity:
Belongs to the ELO family.
Database links:Entrez Gene: 6785Human
Entrez Gene: 83603Mouse
Omim: 605512Human
SwissProt: Q9GZR5Human
SwissProt: Q9EQC4Mouse
SwissProt: Q920L7Rat
Unigene: 101915Human
Unigene: 83949Mouse
Unigene: 7567Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.