Molecular weight (DA)
41kD
Immunogen
Synthesized peptide derived from human PEX14 Polyclonal
Specificity
This antibody detects endogenous levels of PEX14.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB 1:500-2000;IHC-p 1:50-300
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
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],
Function
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
Protein name
Peroxisomal membrane protein PEX14 (PTS1 receptor-docking protein) (Peroxin-14) (Peroxisomal membrane anchor protein PEX14)
Other name
Peroxisomal membrane protein PEX14 (PTS1 receptor-docking protein) (Peroxin-14) (Peroxisomal membrane anchor protein PEX14)
Human protein sequence Database
O75381
Mouse protein sequence database
Q9R0A0
Rat protein sequence database
Q642G4
Cellular localization
Peroxisome membrane ; Peripheral membrane protein ; Cytoplasmic side .
Tissue expression
Brain,Cerebellum,Epithelium,Muscle,Placenta,Testis,
Contract Us
Follow Us
Location： 
Specification
成功添加到购物车