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| Reactivity: | Human, Mouse, Rat |
| Applications: | WB, IF/IC, ELISA |
| Host Species: | Rabbit |
| Isotype: | IgG |
| Clonality: | Polyclonal antibody |
| Gene Name: | peroxisomal biogenesis factor 5 |
| Gene Symbol: | PEX5 |
| Synonyms: | PXR1; PBD2A; PBD2B; PTS1R; RCDP5; PTS1-BP; PEX5 |
| Gene ID: | 5830 |
| UniProt ID: | P50542 |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 364-631 of human PEX5 (NP_000310.2). |
| Dilution: | WB 1:500-1:2000; IF/IC 1:50-1:200 |
| Purification Method: | Affinity purification |
| Concentration: | 2.13 mg/ml |
| Buffer: | PBS with 0.01% thimerosal, 50% glycerol, pH7.3. |
| Storage: | Store at -20°C. Avoid freeze/thaw cycles. |
| Documents: | Manual-PEX5 antibody |
Background
The product of this gene binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. 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 neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD). Alternatively spliced transcript variants encoding different isoforms have been identified.
Images
 | Western blot analysis of various lysates using PEX5 Rabbit pAb (A12493) at 1:1000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 10s. |
 | Immunofluorescence analysis of NIH/3T3 cells using PEX5 Rabbit pAb (A12493) at dilution of 1:100 (40x lens). Secondary antibody: Cy3-conjugated Goat anti-Rabbit IgG (H+L) (AS007) at 1:500 dilution. Blue: DAPI for nuclear staining. |
 | Immunofluorescence analysis of PC-12 cells using PEX5 Rabbit pAb (A12493) at dilution of 1:100 (40x lens). Secondary antibody: Cy3-conjugated Goat anti-Rabbit IgG (H+L) (AS007) at 1:500 dilution. Blue: DAPI for nuclear staining. |
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