Rabbit PEX5 Polyclonal Antibody | anti-PEX5 antibody

PEX5 Antibody

Cat. Num. AAA2527196

• Gene Names: PEX5; PXR1; PBD2A; PBD2B; PTS1R; PTS1-BP
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry
• Purity: Antigen affinity purification
• Synonyms: PEX5; Polyclonal Antibody; PEX5 Antibody; PXR1; PBD2A; PBD2B; PTS1R; PTS1-BP; anti-PEX5 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Antigen affinity purification
• Concentration: 1mg/mL (varies by lot)
• Sequence Length: 660

• Applicable Applications for anti-PEX5 antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Immunogen: Recombinant protein of human PEX5
• Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3
• Santa Cruz Alternative: Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-23188 / sc-23190
• Preparation and Storage: Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.

Western Blot (WB)

(Western blot analysis of extracts of various cell lines, using PEX5 antibody.)

Related Product Information for anti-PEX5 antibody

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.

NCBI and Uniprot Product Information

• NCBI GI #: 663856631
• NCBI GeneID: 5830
• NCBI Accession #: NP_001287718.1
• NCBI GenBank Nucleotide #: NM_001300789.1
• Molecular Weight: 72,291 Da
• NCBI Official Full Name: peroxisomal biogenesis factor 5 isoform e
• NCBI Official Synonym Full Names: peroxisomal biogenesis factor 5
• NCBI Official Symbol: PEX5
• NCBI Official Synonym Symbols: PXR1; PBD2A; PBD2B; PTS1R; PTS1-BP
• NCBI Protein Information: peroxisomal biogenesis factor 5; PTS1 receptor; peroxin-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 receptor 1
• UniProt Protein Name: Peroxisomal targeting signal 1 receptor
• Protein Family: PEX5-related protein
• UniProt Gene Name: PEX5
• UniProt Synonym Gene Names: PXR1; PTS1 receptor; PTS1R
• UniProt Entry Name: PEX5_HUMAN

NCBI Description

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, Oct 2008]

Uniprot Description

PEX5: Binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. Defects in PEX5 are a cause of adrenoleukodystrophy neonatal (NALD). NALD is a peroxisome biogenesis disorder (PBD) characterized by the accumulation of very long- chain fatty acids, adrenal insufficiency and mental retardation. Inheritance is autosomal recessive. Defects in PEX5 are a cause of Zellweger syndrome (ZWS). ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life. Defects in PEX5 may be a cause of infantile Refsum disease (IRD). IRD is a mild peroxisome biogenesis disorder (PBD). Clinical features include early onset, mental retardation, minor facial dysmorphism, retinopathy, sensorineural hearing deficit, hepatomegaly, osteoporosis, failure to thrive, and hypocholesterolemia. The biochemical abnormalities include accumulation of phytanic acid, very long chain fatty acids (VLCFA), di- and trihydroxycholestanoic acid and pipecolic acid. Belongs to the peroxisomal targeting signal receptor family. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Receptor, protein translocating

Chromosomal Location of Human Ortholog: 12p13.31

Cellular Component: peroxisomal membrane; Golgi apparatus; peroxisomal matrix; protein complex; membrane; cytoplasm; peroxisome; intracellular; cytosol

Molecular Function: small GTPase binding; protein C-terminus binding; protein binding; peroxisome targeting sequence binding; enzyme binding; peroxisome matrix targeting signal-1 binding; protein N-terminus binding

Biological Process: protein targeting to peroxisome; protein import into peroxisome matrix; protein import into peroxisome matrix, docking; protein import into peroxisome matrix, translocation; protein import into peroxisome membrane; protein tetramerization

Disease: Peroxisome Biogenesis Disorder 2a (zellweger); Peroxisome Biogenesis Disorder 2b