peroxisomal biogenesis factor 26, ELISA Kit

Mouse Peroxisome assembly protein 26 (PEX26) ELISA kit; FLJ20695; PEX26M1T; Pex26pM1T; peroxin-26; peroxisome assembly protein 26; peroxisome biogenesis disorder; complementation group 8; peroxisome biogenesis disorder; complementation group A; peroxisome bi; peroxisomal biogenesis factor 26

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 PEX26 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.

MBS9324611 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the peroxisomal biogenesis factor 26 (PEX26) 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 PEX26. The ELISA analytical biochemical technique of the MBS9324611 kit is based on PEX26 antibody-PEX26 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect PEX26 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, PEX26. 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(%).

34,016 Da

peroxisomal biogenesis factor 26

peroxisome assembly protein 26; peroxin-26; peroxisome biogenesis factor 26

Peroxisome assembly protein 26

PEX26_MOUSE

This gene is a member of the peroxin-26 family. The encoded protein is probably required for protein import into peroxisomes. It may anchor Pex1 and Pex6 to peroxisome membranes. Defects in a similar gene in human are the cause of peroxisome biogenesis disorder complementation group 8 (PBD-CG8). PBD refers to a group of four disorders: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). Alternatively spliced transcript variants have been identified for this gene. [provided by RefSeq, Feb 2015]

PEX26: Probably required for protein import into peroxisomes. Anchors PEX1 and PEX6 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes. Involved in the import of catalase and proteins containing a PTS2 target sequence, but not in import of proteins with a PTS1 target sequence. Defects in PEX26 are the cause of peroxisome biogenesis disorder complementation group 8 (PBD-CG8); also known as PBD-CGA. PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical continuum of overlapping phenotypes known as the Zellweger spectrum. The PBD group is genetically heterogeneous with at least 13 distinct genetic groups as concluded from complementation studies. Defects in PEX26 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 PEX26 are a cause of adrenoleukodystrophy neonatal (NALD). NALD is a peroxisome biogenesis disorder characterized by the accumulation of very long-chain fatty acids, adrenal insufficiency and mental retardation. Defects in PEX26 are a cause of infantile Refsum disease (IRD). IRD is a mild peroxisome biogenesis disorder. 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 peroxin-26 family.

Protein type: Membrane protein, integral

Cellular Component: integral to peroxisomal membrane; membrane; integral to membrane; peroxisome

Molecular Function: protein C-terminus binding; protein complex binding; ATPase binding

Biological Process: protein transport; transport; protein import into peroxisome matrix; protein import into peroxisome membrane

Disease: Peroxisome Biogenesis Disorder 2b

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