BD-PT3670

Peroxin 10 Rabbit Polyclonal Antibody

PEX10; RNF69; Peroxisome biogenesis factor 10; Peroxin-10; Peroxisomal biogenesis factor 10; Peroxisome assembly protein 10; RING finger protein 69

常规抗体

PEX10

Peroxisome biogenesis factor 10

WB

Human,Rat,Mouse

1 mg/ml

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.

5192

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=5192

O60683

http://www.uniprot.org/uniprotkb/O60683/entry

B1AUE5

http://www.uniprot.org/uniprot/B1AUE5

The antiserum was produced against synthesized peptide derived from human PEX10. AA range:183-232

Peroxin 10 Polyclonal Antibody detects endogenous levels of Peroxin 10 protein.

WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200

45kD

-20°C/1 year

Polyclonal, Rabbit,IgG

peroxisomal biogenesis factor 10(PEX10) Homo sapiens This gene encodes a protein involved in import of peroxisomal matrix proteins. This protein localizes to the peroxisomal membrane. Mutations in this gene result in phenotypes within the Zellweger spectrum of peroxisomal biogenesis disorders, ranging from neonatal adrenoleukodystrophy to Zellweger syndrome. Alternative splicing results in two transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008],

Brain,Lung,

Peroxisome membrane ; Peripheral membrane protein .

disease:Defects in PEX10 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 PEX10 are a cause of Zellweger syndrome (ZWS) [MIM:214100]. 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.,disease:Defects in PEX10 are the cause of peroxisome biogenesis disorder complementation group 7 (PBD-CG7) [MIM:602859]; also known as PBD-CGB. 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 14 distinct genetic groups as concluded from complementation studies.,function:Somewhat implicated in the biogenesis of peroxisomes.,similarity:Belongs to the pex2/pex10/pex12 family.,similarity:Contains 1 RING-type zinc finger.,subunit:Interacts with PEX19.,

现货

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