过氧化物酶体生物合成因子6抗体
规格：1mg/1ml
英文名： PEX6
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.
分子量： 104kDa
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 PEX6
交叉反应：Human, Mouse, Rat,
细胞定位：细胞浆
过氧化物酶体生物合成因子6抗体产品介绍：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. 过氧化物酶体生物合成因子6抗体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),过氧化物酶体生物合成因子6抗体 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: 5190 Human Entrez Gene: 224824 Mouse Entrez Gene: 117265 Rat Omim: 601498 Human SwissProt: Q13608 Human SwissProt: Q99LC9 Mouse SwissProt: P54777 Rat Unigene: 656425 Human Unigene: 299399 Mouse Unigene: 10675 Rat 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6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