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PEX13 Polyclonal Antibody
PEX13 Polyclonal Antibody
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PEX13 Polyclonal Antibody
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经销商客户: ¥440.0
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商品属性

Target:PEX13

Fields:Peroxisome

Gene Name:PEX13

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

Human Gene Id:5194

Human Swiss Prot No:Q92968

Mouse Swiss Prot No:Q9D0K1

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

Specificity:PEX13 Polyclonal Antibody detects endogenous levels of protein.

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

Source:Polyclonal, Rabbit,IgG

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

Storage Stability:-15°C to -25°C/1 year(Do not lower than -25°C)

Observed Band(KD):44kD

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],

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 .

Expression: Liver,

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