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PEX5 Antibody - middle region: FITC (ARP56102_P050-FITC)

Product Specifications

Gene Name

Peroxisomal biogenesis factor 5

Gene Aliases

PXR1, PBD2A, PBD2B, PTS1R, RCDP5, PTS1-BP

Gene ID

5830

Swiss Prot

P50542-3

Accession Number

NP_000310

Host

Rabbit

Reactivity

Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Pig, Rabbit

Immunogen

The immunogen is a synthetic peptide directed towards the middle region of human PEX5

Target

PEX5 binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD) .

Partner Proteins

CAPRIN2; CCDC14; TOMM7; PRR13; GDPD5; ZNF772; EP400NL; TM6SF1; ACOT8; RPL14; MKRN3; S100A6; LDHB; RANBP6; GSTK1; ZADH2; TYSND1; HSDL2; ACAD11; LONP2; PECR; HSD17B4; HOXA7; EHHADH; ECH1; CRAT; CAT; BRCA1; ACOX1; DECR2; HACL1; DHRS4; ECI2; AGPS; GNPAT; UBC

Clonality

Polyclonal

Conjugation

FITC: Fluorescein Isothiocyanate

Type

Polyclonal Antibody

Applications

WB, IHC

Purification

Affinity Purified

Concentration

0.5 mg/mL

Homology

Cow: 100%; Dog: 100%; Guinea Pig: 93%; Horse: 100%; Human: 100%; Mouse: 100%; Pig: 100%; Rabbit: 100%; Rat: 100%

Format

Liquid. Purified antibody supplied in 1x PBS buffer.

Reconstitution

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

Molecular Weight

70kDa

Shipping Conditions

Wet Ice

Protein Length

631

NCBI Gene Symbol

PEX5

Protein Name

Peroxisomal targeting signal 1 receptor

Nucleotide Accession Number

NM_000319

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