Human N-sulphoglucosamine sulphohydrolase, SGSH ELISA Kit | MyBioSource MBS932877 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human N-sulphoglucosamine sulphohydrolase, SGSH ELISA Kit

catalog :

MBS932877

quantity :

48-Strip-Wells

price :

510 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS932877

products type :

ELISA Kit

products full name :

Human N-sulphoglucosamine sulphohydrolase, SGSH ELISA Kit

products short name :

N-sulfoglucosamine sulfohydrolase

products name syn :

Human N-sulphoglucosamine sulphohydrolase (SGSH) ELISA kit; HSS; MPS3A; SFMD; heparan sulfate sulfatase; sulfamidase; sulfoglucosamine sulfamidase; N-sulfoglucosamine sulfohydrolase

other names :

N-sulphoglucosamine sulphohydrolase; N-sulphoglucosamine sulphohydrolase; N-sulphoglucosamine sulphohydrolase; sulphamidase; heparan sulfate sulfatase; sulfoglucosamine sulfamidase; mucopolysaccharidosis type IIIA; N-sulfoglucosamine sulfohydrolase; Sulfoglucosamine sulfamidase; Sulphamidase

products gene name :

SGSH

other gene names :

SGSH; SGSH; HSS; SFMD; MPS3A; HSS

uniprot entry name :

SPHM_HUMAN

reactivity :

Human

sequence length :

502

specificity :

This assay has high sensitivity and excellent specificity for detection of human SGSH. No significant cross-reactivity or interference between human SGSH and analogues was observed.

storage stability :

Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.

other info1 :

Samples: Serum, plasma, tissue homogenates, cell lysates. Assay Type: Sandwich. Detection Range: 31.25 pg/ml-2000 pg/ml. Sensitivity: 7.81 pg /ml.

other info2 :

Intra-assay Precision: Intra-assay Precision (Precision within an assay): CV%<8%. Three samples of known concentration were tested twenty times on one plate to assess. Inter-assay Precision: Inter-assay Precision (Precision between assays): CV%<10%. Three samples of known concentration were tested in twenty assays to assess.

products description :

Principle of the Assay This assay employs the quantitative sandwich enzyme immunoassay technique. Antibody specific for SGSH has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any SGSH present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for SGSH is added to the wells. After washing, avidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of SGSH bound in the initial step. The color development is stopped and the intensity of the color is measured.

ncbi gi num :

4506919

ncbi acc num :

NP_000190.1

ncbi gb acc num :

NM_000199.3

uniprot acc num :

P51688

ncbi mol weight :

56,695 Da

ncbi pathways :

Disease Pathway (530764); Glycogen Storage Diseases Pathway (980468); Glycosaminoglycan Degradation Pathway (82981); Glycosaminoglycan Degradation Pathway (355); Glycosaminoglycan Metabolism Pathway (645297); HS-GAG Degradation Pathway (645307); Heparan Sulfate Degradation Pathway (413377); Heparan Sulfate Degradation Pathway (468270); Heparan Sulfate/heparin (HS-GAG) Metabolism Pathway (645304); Lysosome Pathway (99052)

ncbi summary :

This gene encodes one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with Sanfilippo syndrome A, one type of the lysosomal storage disease mucopolysaccaridosis III, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined. [provided by RefSeq, Jul 2008]

uniprot summary :

SGSH: Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A); also known as Sanfilippo syndrome A. MPS3A is a severe 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. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival. Belongs to the sulfatase family. Protein type: Glycan Metabolism - glycosaminoglycan degradation; Hydrolase; EC 3.10.1.1. Chromosomal Location of Human Ortholog: 17q25.3. Cellular Component: lysosomal lumen. Molecular Function: N-sulfoglucosamine sulfohydrolase activity; metal ion binding; sulfuric ester hydrolase activity; catalytic activity. Biological Process: proteoglycan metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis. Disease: Mucopolysaccharidosis, Type Iiia

size1 :

48-Strip-Wells

price1 :

510 USD

size2 :

96-Strip-Wells

price2 :

725

size3 :

5x96-Strip-Wells

price3 :

2565

size4 :

10x96-Strip-Wells

price4 :

4800

more info or order :

MyBioSource product webpage