PEX5 Rabbit Polyclonal Antibody
CNY 1,999.00
CNY 2,700.00
CNY 3,080.00
CNY 300.00
CNY 1,430.00
CNY 2,900.00
CNY 6,650.00
CNY 9,998.00
Specifications
Product Data | |
Applications | ICC/IF, IHC, WB |
Recommend Dilution | WB 1:500 - 1:2000 |
Reactivity | Human, Mouse, Rat |
Host | Rabbit |
Clonality | Polyclonal |
Immunogen | Recombinant protein of human PEX5 |
Formulation | Store at -20°C (regular) and -80°C (long term). Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Concentration | lot specific |
Purification | Affinity purification |
Conjugation | Unconjugated |
Storage Condition | Store at -20°C as received. |
Predicted Protein Size | 70 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. |
Synonyms | PBD2A; PBD2B; PTS1-BP; PTS1R; PXR1; RCDP5 |
Reference Data | |
Protein Families | Druggable Genome |
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