BD-PN1034

PEX16 Rabbit Polyclonal Antibody

常规抗体

PEX16

Peroxisomal membrane protein PEX16 (Peroxin-16) (Peroxisomal biogenesis factor 16)

WB

Human,Mouse

1 mg/ml

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

9409

Q9Y5Y5

https://www.uniprot.org/uniprotkb/Q9Y5Y5/entry

Q91XC9

http://www.uniprot.org/uniprot/Q91XC9

Synthesized peptide derived from human protein . at AA range: 100-180

PEX16 Polyclonal Antibody detects endogenous levels of protein.

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

36kD

-20°C/1 year

Polyclonal, Rabbit,IgG

peroxisomal biogenesis factor 16(PEX16) Homo sapiens The protein encoded by this gene is an integral peroxisomal membrane protein. An inactivating nonsense mutation localized to this gene was observed in a patient with Zellweger syndrome of the complementation group CGD/CG9. Expression of this gene product morphologically and biochemically restores the formation of new peroxisomes, suggesting a role in peroxisome organization and biogenesis. Alternative splicing has been observed for this gene and two variants have been described. [provided by RefSeq, Jul 2008],

Lung,

Peroxisome membrane ; Multi-pass membrane protein .

disease:Defects in PEX16 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 PEX16 are the cause of peroxisome biogenesis disorder complementation group 9 (PBD-CG9) [MIM:603360]; also known as PBD-CGD. 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:Required for peroxisome membrane biogenesis. May play a role in early stages of peroxisome assembly. Can recruit other peroxisomal proteins, such as PEX3 and PMP34, to de novo peroxisomes derived from the endoplasmic reticulum (ER). May function as receptor for PEX3.,similarity:Belongs to the peroxin-16 family.,subunit:Interacts with PEX19.,

现货

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