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货号： bs-12621R-PE-Cy5.5 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-12621R-PE-Cy5.5
- 英文名称
- Anti-PEX14/PE-Cy5.5
- 中文名称
- PE-Cy5.5标记的过氧化物酶体生物合成因子14抗体
- 别 名
- dJ734G22.2; NAPP2; NF E2 associated polypeptide 2; Peroxin 14; Peroxin-14; Peroxisomal biogenesis factor 14; Peroxisomal membrane anchor protein PEX14; Peroxisomal membrane anchor protein Pex14p; Peroxisomal membrane protein PEX14; pex14; PEX14_HUMAN; Pex14p; PTS1 receptor docking protein; PTS1 receptor-docking protein.
- 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 转录调节因子 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
- 41kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human PEX14
- 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 an essential component of the peroxisomal import machinery. The protein is integrated into peroxisome membranes with its C-terminus exposed to the cytosol, and interacts with the cytosolic receptor for proteins containing a PTS1 peroxisomal targeting signal. The protein also functions as a transcriptional corepressor and interacts with a histone deacetylase. A mutation in this gene results in one form of Zellweger syndrome. [provided by RefSeq, Jul 2008]
Function:
Component of the peroxisomal translocation machinery with PEX13 and PEX17. Interacts with both the PTS1 and PTS2 receptors. Binds directly to PEX17.
Subcellular Location:
Peroxisome membrane.
DISEASE:
Defects in PEX14 are the cause of peroxisome biogenesis disorder complementation group K (PBD-CGK) [MIM:601791]. PBD-CGK is a peroxisomal disorder arising from a failure of protein import into the peroxisomal membrane or matrix. The peroxisome biogenesis disorders (PBD group) are genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Include disorders are: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical continuum of overlapping phenotypes known as the Zellweger spectrum (PBD-ZSS).Defects in PEX14 are a cause of Zellweger syndrome (ZWS) [MIM:214100]. ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life.
Similarity:
Belongs to the peroxin-14 family.
Database links:Entrez Gene: 5195Human
Entrez Gene: 56273Mouse
Entrez Gene: 64460Rat
Omim: 601791Human
SwissProt: O75381Human
SwissProt: Q9R0A0Mouse
SwissProt: Q642G4Rat
Unigene: 149983Human
Unigene: 184172Mouse
Unigene: 7844Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.