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货号： bs-12625R-AF350 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-12625R-AF350
- 英文名称
- Anti-PEX6/AF350
- 中文名称
- AF350标记的过氧化物酶体生物合成因子6抗体
- 别 名
- PAF 2; PAF2; PBD4A; PDB4B; Peroxin 6; Peroxisomal AAA type ATPase 1; Peroxisomal biogenesis factor 6; Peroxisomal type ATPase 1; Peroxisome assembly factor 2; PXAAA1.
- 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 信号转导
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Pig, 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
- 104kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human PEX6
- 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 AAA (ATPases associated with diverse cellular activities) family of ATPases. This member is a predominantly cytoplasmic protein, which plays a direct role in peroxisomal protein import and is required for PTS1 (peroxisomal targeting signal 1, a C-terminal tripeptide of the sequence ser-lys-leu) receptor activity. Mutations in this gene cause peroxisome biogenesis disorders of complementation group 4 and complementation group 6. [provided by RefSeq, Oct 2008]
Function:
Involved in peroxisome biosynthesis. Required for stability of the PTS1 receptor. Anchored by PEX26 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes.
Subcellular Location:
Cytoplasm. Peroxisome membrane. Note: Associated with peroxisomal membranes.
DISEASE:
The disease is caused by mutations affecting the gene represented in this entry.
Disease description: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).
Database links:Entrez Gene: 5190Human
Entrez Gene: 224824Mouse
Entrez Gene: 117265Rat
Omim: 601498Human
SwissProt: Q13608Human
SwissProt: Q99LC9Mouse
SwissProt: P54777Rat
Unigene: 656425Human
Unigene: 299399Mouse
Unigene: 10675Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.