PEX7 Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech

Rabbit Polyclonal Antibody

Supplier:  Proteintech Group Inc 206141AP150UL

Catalog No. 50-173-2988

Description

This gene encodes the cytosolic receptor for the set of peroxisomal matrix enzymes targeted to the organelle by the peroxisome targeting signal 2 (PTS2). Defects in this gene cause peroxisome biogenesis disorders (PBDs), which are characterized by multiple defects in peroxisome function. There are at least 14 complementation groups for PBDs, with more than one 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 have been associated with PBD complementation group 11 (PBD-CG11) disorders, rhizomelic chondrodysplasia punctata type 1 (RCDP1), and Refsum disease (RD).

Specifications

• Antigen: PEX7
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: PEX7
• Gene Alias: Peroxin 7, PEX7, PTS2 receptor, PTS2R, RCDP1, RD
• Host Species: Rabbit
• Purification Method: Antigen Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 18634, 308718, 5191
• Content And Storage: -20°C
• Form: Liquid

• Applications: Western Blot
• Concentration: 0.45 mg/mL
• Formulation: PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
• Gene Accession No.: O00628, P97865
• Gene Symbols: Pex7
• Immunogen: PEX7 Fusion Protein Ag14666
• Quantity: 150 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG