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Recommended positive controls: A431.
Predicted reactivity: Mouse (91%), Bovine (97%), Guinea pig (97%).
Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
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.
⚠WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm. For more information go to www.P65Warnings.ca.gov.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
蛋白别名: peroxin 5; Peroxin-5; peroxisomal biogenesis factor 5; Peroxisomal C-terminal targeting signal import receptor; peroxisomal targeting signal 1 (SKL type) receptor; Peroxisomal targeting signal 1 receptor; peroxisomal targeting signal import receptor; peroxisomal targeting signal receptor 1; peroxisome biogenesis factor 5; Peroxisome receptor 1; PTS1 BP; PTS1 receptor; PTS1-BP; PXR1P
基因别名: AW212715; ESTM1; PBD2A; PBD2B; PEX5; PTS1-BP; PTS1R; PXR1; RCDP5; X83306
UniProt ID: (Human) P50542, (Mouse) O09012
Entrez Gene ID: (Human) 5830, (Mouse) 19305