Human Peroxisomal targeting signal 1 receptor (PEX5) ELISA kit; FLJ50634; FLJ50721; FLJ51948; PTS1-BP; PTS1R; PXR1; peroxin-5; peroxisomal C-terminal targeting signal import receptor; peroxisomal targeting signal 1 (SKL type) receptor; peroxisomal targeting signal; peroxisomal biogenesis factor 5

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of PEX5 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9335740 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the peroxisomal biogenesis factor 5 (PEX5) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing PEX5. The ELISA analytical biochemical technique of the MBS9335740 kit is based on PEX5 antibody-PEX5 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect PEX5 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, PEX5. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

70,865 Da

peroxisomal biogenesis factor 5

peroxisomal biogenesis factor 5; peroxin-5; PTS1 receptor; peroxisome receptor 1; peroxisomal targeting signal 1 receptor; peroxisomal targeting signal receptor 1; peroxisomal targeting signal import receptor; peroxisomal targeting signal 1 (SKL type) receptor; peroxisomal C-terminal targeting signal import receptor

Peroxisomal targeting signal 1 receptor

PXR1; PTS1 receptor; PTS1R??[Similar Products]

PEX5_HUMAN

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]

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 membrane; protein import into peroxisome matrix, translocation; protein tetramerization

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

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