Product Description

PEX13 Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES9979-50, ES9979-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

Background: peroxisomal biogenesis factor 13(PEX13) Homo sapiens 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],

Reactivity: Human, Mouse

Host: Rabbit

Applications: WB, ELISA

Clonality: Polyclonal

Immunogen: Synthesized peptide derived from human protein. at AA range: 290-370

Isotype: IgG

Target: PEX13

Specificity: PEX13 Polyclonal Antibody detects endogenous levels of protein.

Dilution: WB 1:500-2000 ELISA 1:5000-20000

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration: 1 mg/ml,

Formlation: Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

Expression: Liver,

Observed Band (KD): 44kD

Fields: Peroxisome

Function: Caution: It is uncertain whether Met-1 or Met-40 is the initiator
Disease: Defects in PEX13 are a cause of adrenoleukodystrophy neonatal (NALD) [MIM: 202370]. NALD is a peroxisome biogenesis disorder (PBD) characterized by the accumulation of very long-chain fatty acids, adrenal insufficiency and mental retardation
Disease: Defects in PEX13 are the cause of peroxisome biogenesis disorder complementation group 13 (PBD-CG13) [MIM: 601789], also known as PBD-CGH. 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

Subcellular Location: Peroxisome membrane, Single-pass membrane protein

Storage and Stability: -20°C/1 year

Gene Name: PEX13

Protein Name: Peroxisomal membrane protein PEX13 (Peroxin-13)

Mouse SwissProt Number: Q9D0K1

Research Use Only