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货号： bs-4392R-AF594 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-4392R-AF594
- 英文名称
- Anti-FACE1/ZMPSTE24/AF594
- 中文名称
- AF594标记的锌金属蛋白酶STE24同源蛋白抗体
- 别 名
- CAAX prenyl protease 1 homolog; FACE-1; FACE1; FACE1_HUMAN; Farnesylated proteins converting enzyme 1; Farnesylated proteins-converting enzyme 1; Prenyl protein specific endoprotease 1; Prenyl protein-specific endoprotease 1; STE24; Zinc metalloproteinase Ste24 homolog; zmpste24.
- 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 发育生物学 转录调节因子 锌指蛋白 表观遗传学
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, Sheep,
- 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
- 55kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human FACE1/ZMPSTE24
- 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 peptidase M48A family. The encoded protein is a zinc metalloproteinase involved in the two step post-translational proteolytic cleavage of carboxy terminal residues of farnesylated prelamin A to form mature lamin A. Mutations in this gene have been associated with mandibuloacral dysplasia and restrictive dermopathy. [provided by RefSeq, Jul 2008]
Function:
Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C.
Subcellular Location:
Endoplasmic reticulum membrane. Golgi apparatus membrane.
Tissue Specificity:
Widely expressed. High levels in kidney, prostate, testis and ovary.
DISEASE:
Defects in ZMPSTE24 are the cause of mandibuloacral dysplasia with type B lipodystrophy (MADB) [MIM:608612]. Mandibuloacral dysplasia (MAD) is a rare autosomal recessive disorder characterized by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of the cranial suture, joint contractures, and types A or B patterns of lipodystrophy. Type B lipodystrophy observed in MADB, is characterized by generalized fat loss.
Defects in ZMPSTE24 are a cause of lethal tight skin contracture syndrome (LTSCS) [MIM:275210]; also called restrictive dermopathy (RD). Lethal tight skin contracture syndrome is a rare disorder mainly characterized by intrauterine growth retardation, tight and rigid skin with erosions, prominent superficial vasculature and epidermal hyperkeratosis, facial features (small mouth, small pinched nose and micrognathia), sparse/absent eyelashes and eyebrows, mineralization defects of the skull, thin dysplastic clavicles, pulmonary hypoplasia, multiple joint contractures and an early neonatal lethal course. Liveborn children usually die within the first week of life. The overall prevalence of consanguineous cases suggested an autosomal recessive inheritance.
Similarity:
Belongs to the peptidase M48A family.
Database links:Entrez Gene: 10269Human
Omim: 606480Human
SwissProt: O75844Human
Unigene: 132642Human
Unigene: 721062Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.