84-5554-95　［取扱停止］Peroxin 5 30ul　540912

特徴

• The product of PEX5 (peroxisomal biogenesis factor 5) 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 PEX5 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.
• Applications:Suitable for use in Western Blot and ELISA.
• Other applications not tested.
• Recommended Dilution:Western Blot: 1:500-1:2000ELISA: 1:40,000Optimal dilutions to be determined by the researcher.
• Storage and Stability:May be stored at 4°C for short-term only.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Aliquots are stable for 12 months after receipt.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.