85-6366-92　Peroxin 5 Polyclonal Antibody 100ul　BT-AP07076

特徴

• 背景：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.

仕様

• サイズ：100ul
• 抗原：Synthesized peptide derived from Peroxin 5 . at AA range: 540-620
• 免疫動物：Rabbit
• 交差性：Human;Mouse
• 適用：WB, ELISA
• クラス：IgG
• クロナリティ：Polyclonal
• 濃度：1 mg/ml
• 別名：PEX5; PXR1; Peroxisomal targeting signal 1 receptor; PTS1 receptor; PTS1R; PTS1-BP; Peroxin-5; Peroxisomal C-terminal targeting signal import receptor; Peroxisome receptor 1
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