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货号： bs-12624R-AF350 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-12624R-AF350
- 英文名称
- Anti-PEX5/AF350
- 中文名称
- AF350标记的过氧化物酶体生物合成因子5抗体
- 别 名
- FLJ50634; FLJ50721; FLJ51948; Peroxin 5; Peroxin-5; Peroxisomal biogenesis factor 5; Peroxisomal C terminal targeting signal import receptor; Peroxisomal C-terminal targeting signal import receptor; Peroxisomal targeting signal 1 receptor; Peroxisome receptor 1; pex5; PEX5_HUMAN; PTS1 BP; PTS1 receptor; PTS1-BP; PTS1R; PXR1.
- 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 信号转导 转录调节因子
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Horse, 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
- 71kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human PEX5
- 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:
The product of this gene binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD). Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Oct 2008]
Function:
Binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import.
Subcellular Location:
Cytoplasm. Peroxisome membrane. Its distribution appears to be dynamic. It is probably a cycling receptor found mainly in the cytoplasm and as well associated to the peroxisomal membrane through a docking factor.
Tissue Specificity:
Detected in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.
DISEASE:
Defects in PEX5 are a cause of adrenoleukodystrophy neonatal (NALD) [MIM:202370]. NALD is a peroxisome biogenesis disorder (PBD) characterized by the accumulation of very long-chain fatty acids, adrenal insufficiency and mental retardation. Inheritance is autosomal recessive.
Defects in PEX5 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.
Defects in PEX5 may be a cause of infantile Refsum disease (IRD) [MIM:266510]. IRD is a mild peroxisome biogenesis disorder (PBD). Clinical features include early onset, mental retardation, minor facial dysmorphism, retinopathy, sensorineural hearing deficit, hepatomegaly, osteoporosis, failure to thrive, and hypocholesterolemia. The biochemical abnormalities include accumulation of phytanic acid, very long chain fatty acids (VLCFA), di- and trihydroxycholestanoic acid and pipecolic acid.
Similarity:
Belongs to the peroxisomal targeting signal receptor family.
Contains 7 TPR repeats.
Database links:Entrez Gene: 418299Chicken
Entrez Gene: 514832Cow
Entrez Gene: 486710Dog
Entrez Gene: 100135597 Guinea pig
Entrez Gene: 5830Human
Entrez Gene: 19305Mouse
Entrez Gene: 312703Rat
Omim: 600414Human
SwissProt: Q5ZMQ9Chicken
SwissProt: Q1RMV0Cow
SwissProt: O70525 Guinea pig
SwissProt: P50542Human
SwissProt: O09012Mouse
SwissProt: Q2M2R8Rat
Unigene: 19020Chicken
Unigene: 10901Cow
Unigene: 567327Human
Unigene: 22418Mouse
Unigene: 14316Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.