FITC标记的过氧化物酶体生物合成因子10抗体-抗体-抗体-生物在线
上海沪震实业有限公司
FITC标记的过氧化物酶体生物合成因子10抗体

FITC标记的过氧化物酶体生物合成因子10抗体

商家询价

产品名称: FITC标记的过氧化物酶体生物合成因子10抗体

英文名称: Anti-PEX10/FITC

产品编号: HZ--0355R-FITC

产品价格: null

产品产地: 中国/上海

品牌商标: HZbscience

更新时间: 2023-08-17T10:24:20

使用范围: ICC=1:50-200 IF=1:50-200

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 Rabbit Anti-PEX10/FITC Conjugated antibody

FITC标记的过氧化物酶体生物合成因子10抗体

 

英文名称 Anti-PEX10/FITC
中文名称 FITC标记的过氧化物酶体生物合成因子10抗体
别    名 AV128229; Gm142; MGC1998; NALD; OTTHUMP00000001658; PBD6A; PBD6B; peroxin 10; Peroxin-10; Peroxisomal biogenesis factor 10; Peroxisome assembly protein 10; Peroxisome biogenesis factor 10; PEX10; PEX10_HUMAN; RING finger protein 69; RNF69; RP23-298E4.1.  
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, Cow, 
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 PEX10
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 protein involved in import of peroxisomal matrix proteins. This protein localizes to the peroxisomal membrane. Mutations in this gene result in phenotypes within the Zellweger spectrum of peroxisomal biogenesis disorders, ranging from neonatal adrenoleukodystrophy to Zellweger syndrome. Alternative splicing results in two transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]

Function:
Somewhat implicated in the biogenesis of peroxisomes.

Subcellular Location:
Peroxisome membrane.

DISEASE:
Peroxisome biogenesis disorder complementation group 7.
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). Peroxisome biogenesis disorder 6A.
The disease is caused by mutations affecting the gene represented in this entry. Disease description:A fatal peroxisome biogenesis disorder belonging to the Zellweger disease spectrum and clinically characterized by severe neurologic dysfunction with profound psychomotor retardation, severe hypotonia and neonatal seizures, craniofacial abnormalities, liver dysfunction, and biochemically by the absence of peroxisomes. Additional features include cardiovascular and skeletal defects, renal cysts, ocular abnormalities, and hearing impairment. Most severely affected individuals with the classic form of the disease (classic Zellweger syndrome) die within the first year of life. Peroxisome biogenesis disorder 6B.
The disease is caused by mutations affecting the gene represented in this entry. Disease description:A peroxisome biogenesis disorder that includes neonatal adrenoleukodystrophy (NALD) and infantile Refsum disease (IRD), two milder manifestations of the Zellweger disease spectrum. The clinical course of patients with the NALD and IRD presentation is variable and may include developmental delay, hypotonia, liver dysfunction, sensorineural hearing loss, retinal dystrophy and vision impairment. Children with the NALD presentation may reach their teens, while patients with the IRD presentation may reach adulthood. The clinical conditions are often slowly progressive in particular with respect to loss of hearing and vision. 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 pex2/pex10/pex12 family.
Contains 1 RING-type zinc finger.

Database links:

Entrez Gene: 5192 Human

Entrez Gene: 668173 Mouse

Entrez Gene: 680424 Rat

Omim: 602859 Human

SwissProt: O60683 Human

SwissProt: B1AUE5 Mouse

Unigene: 732228 Human

Unigene: 133114 Mouse

Unigene: 111 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications
   

该基因编码涉及过氧化物酶体基质蛋白的导入的蛋白质。该蛋白定位为过氧化物酶体膜。该基因的突变导致过氧化物酶体生物发生障碍的齐尔韦格谱内的表型,从新生儿肾上腺脑白质营养不良到齐尔韦格综合征。选择性剪接结果在两个转录变体中编码不同的亚型。