PEX5 Antilichaam - N-terminalgebied: FITC (ARP56101_P050-FITC)

Peroxisomal biogenesis factor 5

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

5830

P50542-3

NP_000310

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

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

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) .

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

FITC: Fluorescein Isothiocyanate

Polyclonal Antibody

WB

Affinity Purified

0.5 mg/ml

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

Liquid. Purified antibody supplied in 1x PBS buffer.

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.

70kDa

Maynard, E.L. (2007) J. Mol. Biol. 368 (5), 1259-1266

Wet Ice

631

PEX5

PEX5

Rabbit

Peroxisomal targeting signal 1 receptor

NM_000319

TATDRWYDEYHPEEDLQHTASDFVAKVDDPKLANSEFLKFVRQIGEGQVS