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PEX13 Polyclonal Antibody

说明书

BYab-05906

  • 20UL ¥460 50UL ¥945 100UL ¥1350
  • 货期: 3天左右

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宿主
反应性
应用
WB;ELISA
分子量(DA)
44kD
免疫原
Synthesized peptide derived from human protein . at AA range: 290-370
特异性
PEX13 Polyclonal Antibody detects endogenous levels of protein.
来源
Polyclonal, Rabbit,IgG
组成(Formulation)
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
稀释比例
WB 1:500-2000 ELISA 1:5000-20000
纯化工艺(Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
浓度
1 mg/ml
背景(Background)
peroxisomal biogenesis factor 13(PEX13) Homo sapiens This gene encodes a peroxisomal membrane protein that binds the type 1 peroxisomal targeting signal receptor via a SH3 domain located in the cytoplasm. Mutations and deficiencies in peroxisomal protein importing and peroxisome assembly lead to peroxisomal biogenesis disorders, an example of which is Zellweger syndrome. [provided by RefSeq, Oct 2008],
功能
caution:It is uncertain whether Met-1 or Met-40 is the initiator.,disease:Defects in PEX13 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.,disease:Defects in PEX13 are the cause of peroxisome biogenesis disorder complementation group 13 (PBD-CG13) [MIM:601789]; also known as PBD-CGH. PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: 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
基因名称(Gene Name)
PEX13
蛋白名称
Peroxisomal membrane protein PEX13 (Peroxin-13)
简称
PEX13
其他名称
Fields
>>Peroxisome
人基因ID
5194
人蛋白质序列数据库
Q92968
小鼠基因ID
小鼠蛋白质序列数据库
Q9D0K1
大鼠基因ID
大鼠蛋白质序列数据库
细胞定位
Peroxisome membrane ; Single-pass membrane protein .
组织表达
Liver,
储存(Storage)
-20°C/1 year

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PEX13 Polyclonal Antibody

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