| ELK.No | ES8932 |
| Product name | PEX14 rabbit pAb |
| Reactivity | Human;Mouse;Rat |
| Applications | WB;IHC |
| Other name | Peroxisomal membrane protein PEX14 (PTS1 receptor-docking protein) (Peroxin-14) (Peroxisomal membrane anchor protein PEX14) |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 5195 |
| Human Swiss-Prot | O75381 |
| Source | Rabbit |
| Isotype | IgG |
| 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 link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 56273 |
| Mouse gene link | |
| Mouse Swiss-Prot | Q9R0A0 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | 64460 |
| Rat gene link | |
| Rat Swiss-Prot | Q642G4 |
| Rat Swiss link | View Rat Swiss-Prot |
| 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. |
| Clonality | Polyclonal |
| Dilution | WB 1:500-2000;IHC-p 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 | -20°C/1 year |
| Molecular Weight (Da) | |
| 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, |


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