GNS Antilichaam - C-terminalgebied: Biotine (ARP45217_P050-Biotine)

Glucosamine (N-acetyl) -6-sulfatase

G6S

2799

P15586

NP_002067

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

The immunogen is a synthetic peptide directed towards the C terminal region of human GNS

GNS is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparin sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder ucopolysaccharidosis type IIID (Sanfilippo D syndrome) . Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome.The product of this gene is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome) . Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome.

LDHAL6B; BCCIP; UBFD1; CNDP2; SCLY; C11orf58; GDA; KYNU; RPS6KA1; PEPD; MVD; GLS; GLA; GBP2; G6PD; EIF5A; CASP7; CAPN1; FBXO6; UBC

Biotin

Polyclonal Antibody

WB, IHC

Affinity Purified

0.5 mg/ml

Cow: 100%; Dog: 93%; Goat: 100%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 100%; Rat: 100%; Zebrafish: 85%

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.

62kDa

Jansen, A.C., (2007) Arch. Neurol. 64 (11), 1629-1634

Wet Ice

552

GNS

GNS

Rabbit

N-acetylglucosamine-6-sulfatase

NM_002076

PILRGASNLTWRSDVLVEYQGEGRNVTDPTCPSLSPGVSQCFPDCVCEDA