产品名称
Peroxin 14 Rabbit Polyclonal Antibody
别名
PEX14; Peroxisomal membrane protein PEX14; PTS1 receptor-docking protein; Peroxin-14; Peroxisomal membrane anchor protein PEX14
蛋白名称
Peroxisomal membrane protein PEX14
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=5195
Human Swissprot No.
O75381
Human Swissprot Link
http://www.uniprot.org/uniprotkb/O75381/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=56273
Mouse Swissprot No.
Q9R0A0
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q9R0A0
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=64460
Rat Swissprot Link
http://www.uniprot.org/uniprot/Q642G4
免疫原
The antiserum was produced against synthesized peptide derived from human PEX14. AA range:117-166
特异性
Peroxin 14 Polyclonal Antibody detects endogenous levels of Peroxin 14 protein.
稀释度
WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:20000.. IF 1:50-200
宿主
Polyclonal, Rabbit,IgG
背景介绍
peroxisomal biogenesis factor 14(PEX14) Homo sapiens This gene encodes an essential component of the peroxisomal import machinery. The protein is integrated into peroxisome membranes with its C-terminus exposed to the cytosol, and interacts with the cytosolic receptor for proteins containing a PTS1 peroxisomal targeting signal. The protein also functions as a transcriptional corepressor and interacts with a histone deacetylase. A mutation in this gene results in one form of Zellweger syndrome. [provided by RefSeq, Jul 2008],
组织表达
Brain,Cerebellum,Epithelium,Muscle,Placenta,Testis,
细胞定位
Peroxisome membrane ; Peripheral membrane protein ; Cytoplasmic side .
功能
disease:Defects in PEX14 are a cause of Zellweger syndrome (ZWS) [MIM:214100]. ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life.,disease:Defects in PEX14 are the cause of peroxisome biogenesis disorder complementation group K (PBD-CGK) [MIM:601791]. 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 as the Zellweger spectrum. The PBD group is genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies.,function:Component of the peroxisomal translocation machinery with PEX13 and PEX17. Interacts with both the PTS1 and PTS2 receptors. Binds directly to PEX17.,similarity:Belongs to the peroxin-14 family.,subunit:Interacts with PEX19.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
