Target：PEX14

Fields：Peroxisome

Gene Name：PEX14

Protein Name：Peroxisomal membrane protein PEX14 (PTS1 receptor-docking protein) (Peroxin-14) (Peroxisomal membrane anchor protein PEX14)

Human Gene Id：5195

Human Swiss Prot No：O75381

Mouse Gene Id：56273

Mouse Swiss Prot No：Q9R0A0

Rat Gene Id：64460

Rat Swiss Prot No：Q642G4

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.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500-2000;IHC 1:50-300

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：Peroxisomal membrane protein PEX14 (PTS1 receptor-docking protein) (Peroxin-14) (Peroxisomal membrane anchor protein PEX14)

Observed Band(KD)：41kD

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

Subcellular Location：Peroxisome membrane ; Peripheral membrane protein ; Cytoplasmic side .

Expression： Brain,Cerebellum,Epithelium,Muscle,Placenta,Testis,