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Antibody Product Overview
| ELK.No | ES9985 |
| Product name | PEX12 rabbit pAb |
| Reactivity | Human;Rat;Mouse |
| Applications | WB;ELISA |
| Other name | |
| Size | 50μL |
| Unit price ($) | 148 |
| Human gene ID | 5193 |
| Human Swiss-Prot | O00623 |
| Source | Rabbit |
| Isotype | IgG |
| Target | PEX12 |
| Fields | >>Peroxisome |
| Gene name | PEX12 PAF3 |
| Protein name | Peroxisome assembly protein 12 (Peroxin-12) (Peroxisome assembly factor 3) (PAF-3) |
| Human gene link | |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | |
| Mouse gene link | |
| Mouse Swiss-Prot | Q8VC48 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | |
| Rat gene link | |
| Rat Swiss-Prot | O88177 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | Synthesized peptide derived from human protein . at AA range: 180-260 |
| Specificity | PEX12 Polyclonal Antibody detects endogenous levels of protein. |
| Formulation | Liquid in PBS containing 50% glycerol, and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1:500-2000 ELISA 1:5000-20000 |
| 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) | 39kD |
| Background | peroxisomal biogenesis factor 12(PEX12) Homo sapiens This gene belongs to the peroxin-12 family. Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of Zellweger syndrome (ZWS). [provided by RefSeq, Oct 2008], |
| Function | disease:Defects in PEX12 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 PEX12 are the cause of peroxisome biogenesis disorder complementation group 3 (PBD-CG3) [MIM:601758]. 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 ; Multi-pass membrane protein . |
| Expression | Fetal brain,Testis, |
Additional Images
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Western blot analysis of lysates from KB cells, primary antibody was diluted at 1:1000, 4°over night
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: AO-06-ES9985-50
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