过氧化物酶体生物合成因子16抗体
规格:1尘驳/1尘濒
英文名: PEX16
别名: Peroxin 16; PBD8A; PBD8B; Peroxin16; Peroxisomal biogenesis factor 16; Peroxisomal membrane protein PEX16; PEX 16.
分子量: 39kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:笔辞濒测肠濒辞苍补濒
亚型:滨驳骋
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human PEX16
交叉反应:贬耻尘补苍,
细胞定位:细胞浆 细胞膜
过氧化物酶体生物合成因子16抗体产物介绍:background: The protein encoded by this gene is an integral peroxisomal membrane protein. An inactivating nonsense mutation localized to this gene was observed in a patient with Zellweger syndrome of the complementation group CGD/CG9. Expression of this gene product morphologically and biochemically restores the formation of new peroxisomes, suggesting a role in peroxisome organization and biogenesis. Alternative splicing has been observed for this gene and two variants have been described. [provided by RefSeq, Jul 2008] Function: Required for peroxisome membrane biogenesis. May play a role in early stages of peroxisome assembly. Can recruit other peroxisomal proteins, such as PEX3 and PMP34, to de novo peroxisomes derived from the endoplasmic reticulum (ER). May 过氧化物酶体生物合成因子16抗体function as receptor for PEX3. Subcellular Location: Peroxisome membrane; Multi-pass membrane protein. Endoplasmic reticulum membrane. 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过氧化物酶体生物合成因子16抗体 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). Similarity: Belongs to the peroxin-16 family. Database links: Entrez Gene: 9409 Human Omim: 603360 Human SwissProt: Q9Y5Y5 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16抗体产物应用: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