PEX5 Mouse Monoclonal Antibody [Clone ID: OTI6E9]
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Other products for "PEX5"
Specifications
Product Data | |
Clone Name | OTI6E9 |
Applications | FC, IF, WB |
Recommended Dilution | WB 1:500~2000, IF 1:100, FLOW 1:100 |
Reactivities | Human, Monkey, Mouse, Rat, Dog |
Host | Mouse |
Isotype | IgG1 |
Clonality | Monoclonal |
Immunogen | Full length human recombinant protein of human PEX5 (NP_000310) produced in HEK293T cell. |
Formulation | PBS (PH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide. |
Concentration | 0.57 mg/ml |
Purification | Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G) |
Conjugation | Unconjugated |
Storage | Store at -20°C as received. |
Stability | Stable for 12 months from date of receipt. |
Predicted Protein Size | 69.7 kDa |
Gene Name | peroxisomal biogenesis factor 5 |
Database Link | |
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. [provided by RefSeq] |
Synonyms | PBD2A; PBD2B; PTS1-BP; PTS1R; PXR1; RCDP5 |
Reference Data | |
Protein Families | Druggable Genome |
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