过氧化物酶体生物合成因子13抗体
规格：1mg/1ml
英文名： PEX13
别名： 2610008O20Rik; NALD; OTTMUSP00000005454; Peroxin 13; Peroxin-13; Peroxin13; Peroxisomal membrane protein PEX 13; Peroxisomal membrane protein PEX13; Peroxisome biogenesis factor 13; PEX 13; pex13; PEX
分子量： 44kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PEX13
交叉反应：Human, Mouse,
细胞定位：细胞浆 细胞膜
过氧化物酶体生物合成因子13抗体产品介绍：background: 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] Function: Component of the peroxisomal translocation machinery with PEX14 and PEX17. Functions as a docking factor for the predominantly cytoplasmic PTS1 receptor (PAS10/PEX5). Involved in the import of PTS1 and PTS2 proteins. Subcellular Location: Peroxisome membrane. DISEASE: Defects in PEX13 are the cause of peroxisome biogenesis disorder complementation group 13 (PBD-CG13) [MIM:601789]; also known as PBD-CGH. PBD-CG13 is a peroxisomal disorder arising from a failure of protein import into the peroxisomal membrane or matrix. The peroxisome过氧化物酶体生物合成因子13抗体 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 PEX13 are a cause of adrenoleukodystrophy neonatal 过氧化物酶体生物合成因子13抗体(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. Similarity: Belongs to the peroxin-13 family. Contains 1 SH3 domain. Database links: Entrez Gene: 5194 Human Omim: 601789 Human SwissProt: Q92968 Human Unigene: 161377 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
过氧化物酶体生物合成因子13抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid