antibody

Goat anti-PEX13 Antibody

MBS420179

0.1 mg

300 USD

polyclonal

goat

nonconjugated

immunohistochemistry, enzyme immunoassay

Antibody

Goat anti-PEX13 Antibody

PEX13

PEX13; peroxisome biogenesis factor 13; ZWS; NALD; peroxisomal biogenesis factor 13; PEX13 antibody; peroxisome biogenesis factor 13 antibody; ZWS antibody; NALD antibody; peroxisomal biogenesis factor 13 antibody

peroxisome biogenesis factor 13; Peroxisomal membrane protein PEX13; peroxisome biogenesis factor 13; peroxisomal biogenesis factor 13; Peroxin-13

PEX13

PEX13; PEX13; ZWS; NALD; PBD11A; PBD11B

PEX13_HUMAN

Polyclonal

Goat

Tested: Human; Expected from sequence similarity: Human

403

DSIGKDGEKQDL

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

100ug specific antibody in 200ul

Aliquot and store at -20 degree C. Minimize freezing and thawing.

Peptide ELISA (EIA), Immunohistochemistry (IHC)

Peptide ELISA: Antibody detection limit dilution 1: 8000. Immunohistochemistry: In paraffin embedded Human Liver shows vesicular staining in the cytoplasm of the hepatocytes. Recommended concentration, 2-4ug/ml.

Immunogen: Peptide with sequence C-DSIGKDGEKQDL, from the C Terminus of the protein sequence according to NP_002609.1. Epitope: C Terminus

4505723

NP_002609.1

NM_002618.3

44,130 Da

Peroxisome Pathway (131226); Peroxisome Pathway (131126)

This gene encodes a peroxisomal membrane protein that binds the type 1 peroxisomal targeting signal receptor via a SH3 domain located in the cytoplasm. Mutations and deficiencies in peroxisomal protein importing and peroxisome assembly lead to peroxisomal biogenesis disorders, an example of which is Zellweger syndrome. [provided by RefSeq, Oct 2008]

PEX13: Component of the peroxisomal translocation machinery with PEX14 and PEX17. Functions as a docking factor for the predominantly cytoplasmic PTS1 receptor (PAS10/PEX5). Involved in the import of PTS1 and PTS2 proteins. Defects in PEX13 are the cause of peroxisome biogenesis disorder complementation group 13 (PBD-CG13); also known as PBD-CGH. PBD-CG13 is a peroxisomal disorder arising from a failure of protein import into the peroxisomal membrane or matrix. The peroxisome biogenesis disorders (PBD group) are genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Include disorders are: 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 (PBD-ZSS). Defects in PEX13 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. Belongs to the peroxin-13 family. Protein type: Membrane protein, integral. Chromosomal Location of Human Ortholog: 2p16.1. Cellular Component: integral to peroxisomal membrane; intracellular membrane-bound organelle; membrane; peroxisomal membrane; peroxisome. Molecular Function: protein binding. Biological Process: cerebral cortex cell migration; fatty acid alpha-oxidation; locomotory behavior; microtubule-based peroxisome localization; neuron migration; positive regulation of defense response to virus by host; protein import into peroxisome matrix, docking; suckling behavior. Disease: Peroxisome Biogenesis Disorder 11a (zellweger); Peroxisome Biogenesis Disorder 11b

0.1 mg

300 USD