GTX109798
PEX5 antibody
Eine Lieferung in diese Region ist nicht möglich.
- SKU:
- GTX109798
- Zusätzliche Namen:
- peroxisomal biogenesis factor 5 , PBD2A , PBD2B , PTS1-BP , PTS1R , PXR1 , RCDP5
- Anwendung:
- WB, IHC-P
- Konzentration:
- 1 mg/ml
- Physischer Zustand:
- Liquid
- Spezies-Reaktivität:
- Human
- Aufreinigung:
- Affinity Purified
- Lagerbedingungen:
- -20[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., 2-8[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., -20[o]C/-70[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted.
- Hersteller:
- Genetex
- Host:
- Rabbit
- Reaktivitäten:
- Human, Mouse
- Buffer:
- 0.1M Tris, 0.1M Glycine, 10% Glycerol, 0.01% Thimerosal.
- Immunogen:
- Recombinant protein encompassing a sequence within the center region of human PEX5. The exact sequence is proprietary.
- Uniprot:
- P50542
- Synonyme:
- PBD2A;PBD2B;peroxin-5;peroxisomal biogenesis factor 5;peroxisomal C-terminal targeting signal import receptor;peroxisomal import receptor 5;peroxisomal targeting signal 1 (SKL type) receptor;peroxisomal targeting signal 1 receptor;peroxisomal targeting signal import receptor;peroxisomal targeting signal receptor 1;peroxisome receptor 1;PTS1 receptor;PTS1-BP;PTS1R;PXR1;RCDP5
- Weitere Details:
- 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]
- Versandbedingungen:
- Blue Ice