ELISA/assay

Human N-acetylglucosamine-6-sulfatase, GNS ELISA Kit

MBS9341333

48-Strip-Wells

470 USD

ELISA Kit

Human N-acetylglucosamine-6-sulfatase, GNS ELISA Kit

[glucosamine (N-acetyl) -6-sulfatase]

[Human N-acetylglucosamine-6-sulfatase (GNS) ELISA kit; G6S; MGC21274; N-acetylglucosamine-6-sulfatase; glucosamine-6-sulfatase; glucosamine (N-acetyl) -6-sulfatase]

[N-acetylglucosamine-6-sulfatase; N-acetylglucosamine-6-sulfatase; N-acetylglucosamine-6-sulfatase; glucosamine (N-acetyl)-6-sulfatase; Glucosamine-6-sulfatase; G6S]

[GNS]

[GNS; GNS; G6S; G6S]

GNS_HUMAN

Human

No significant cross-reactivity or interference between this analyte and analogues is observed.

Store all reagents at 2-8 degree C

Samples: Mouse Body Fluids And Tissue Homogenates. Assay Type: Quantitative Sandwich. Detection Range: 0.25ng/ml-8ng/ml. Sensitivity: 0.1ng/ml.

Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100]. All CV% should be compared by concentration, not compared by OD values.

Background/Introduction: This Quantitative Sandwich ELISA kit is for lab reagent/research use only, not for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determine the level of GNRHR (hereafter termed "analyte") in undiluted original Mouse body fluids and tissue homogenates. This kit is NOT suitable for assaying non-biological sources of substances.

4504061

NP_002067.1

NM_002076.3

P15586

62,082 Da

Clathrin Derived Vesicle Budding Pathway (119545); Glycosaminoglycan Degradation Pathway (82981); Glycosaminoglycan Degradation Pathway (355); Heparan Sulfate Degradation Pathway (413377); Heparan Sulfate Degradation Pathway (468270); Keratan Sulfate Degradation Pathway (413378); Keratan Sulfate Degradation Pathway (468269); Lysosome Pathway (99052); Lysosome Pathway (96865); Lysosome Vesicle Biogenesis Pathway (119547)

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. [provided by RefSeq, Jul 2008]

GNS: Defects in GNS are the cause of mucopolysaccharidosis type 3D (MPS3D); also known as Sanfilippo D syndrome. MPS3D is a form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. Belongs to the sulfatase family. Protein type: Glycan Metabolism - glycosaminoglycan degradation; Hydrolase; EC 3.1.6.14. Chromosomal Location of Human Ortholog: 12q14. Cellular Component: lysosomal lumen. Molecular Function: protein binding; N-acetylglucosamine-6-sulfatase activity; metal ion binding; sulfuric ester hydrolase activity. Biological Process: keratan sulfate metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis; keratan sulfate catabolic process. Disease: Mucopolysaccharidosis, Type Iiid

48-Strip-Wells

470 USD

96-Strip-Wells

680

5x96-Strip-Wells

3100

10x96-Strip-Wells

6095