PEX5 sirna

PEX5 siRNA (Human)

Cat. Num. AAA8229377

• Gene Names: PEX5; PXR1; PBD2A; PBD2B; PTS1R; PTS1-BP
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: PEX5; PEX5 siRNA (Human); PXR1; Peroxisomal targeting signal 1 receptor; PTS1 receptor; PTS1R; PTS1-BP; Peroxin-5; Peroxisomal C-terminal targeting signal import receptor; Peroxisome receptor 1; PEX5 sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: PEX5 siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
• Purity/Purification: > 97%
• Form/Format: Lyophilized powder
• Sequence Length: 631

• Applicable Applications for PEX5 sirna: RNA Interference (RNAi)
• Quality Control: Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
• Directions for Use: We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
• Components: We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human PEX5 gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
• Preparation and Storage: Shipped at 4 degree C. Store at -20 degree C for one year.

Related Product Information for PEX5 sirna

siRNA to inhibit PEX5 expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 21361204
• NCBI GeneID: 5830
• NCBI Accession #: NP_000310.2
• NCBI GenBank Nucleotide #: NM_000319.4
• UniProt Accession #: P50542; Q15115; Q15266; Q96FN7; A8K891; B4DZ45; B7ZAD5; D3DUT8
• Molecular Weight: 72,291 Da
• NCBI Official Full Name: peroxisomal biogenesis factor 5 isoform b
• 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
• 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; enzyme binding; peroxisome targeting sequence 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