Rabbit anti-human SGSH polyclonal Antibody | MyBioSource MBS1497890 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Rabbit anti-human SGSH polyclonal Antibody

catalog :

MBS1497890

quantity :

0.05 mL

price :

120 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human

application :

ELISA, immunohistochemistry, enzyme immunoassay

more info or order :

MyBioSource product webpage

image

image 1 :

Immunohistochemistry of paraffin-embedded human kidney using MBS1497890 at dilution 1:100

image 2 :

Immunohistochemistry of paraffin-embedded human adrenal gland using MBS1497890 at dilution 1:100

product information

catalog number :

MBS1497890

products type :

Antibody

products full name :

Rabbit anti-human SGSH polyclonal Antibody

products short name :

[SGSH]

products name syn :

[Sulfoglucosamine sulfamidase;Sulphamidase;SGSH;HSS]

other names :

[N-sulphoglucosamine sulphohydrolase; N-sulphoglucosamine sulphohydrolase; N-sulphoglucosamine sulphohydrolase; N-sulfoglucosamine sulfohydrolase; Sulfoglucosamine sulfamidase; Sulphamidase]

products gene name :

[SGSH]

other gene names :

[SGSH; SGSH; HSS; SFMD; MPS3A; HSS]

uniprot entry name :

SPHM_HUMAN

clonality :

Polyclonal

isotype :

IgG

host :

Rabbit

reactivity :

Human

sequence length :

502

purity :

Antigen Affinity Purified

storage stability :

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.

tested application :

ELISA (EIA), Immunohistochemistry (IHC)

app notes :

Recommended dilution: IHC:1:20-1:200

image1 heading :

Immunohistochemistry (IHC)

image2 heading :

Immunohistochemistry (IHC)

other info1 :

Storage Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. Conjugate: Non-conjugated

other info2 :

Immunogen: Recombinant human N-sulphoglucosamine sulphohydrolase protein (273-502aa). Santa Cruz Alternative: Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-79741 / sc-79742

products description :

SGSH(N-sulphoglucosamine sulphohydrolase) is also named as HSS, sulphamidase and belongs to the sulfatase family. Sulfamidase is synthesized as a 62 kDa precursor protein, which is modified with mannose 6-phosphate (M6P) residues, allowing their recognition by mannose-6-phosphate receptors in the Golgi complex and ensuring transport to the endosomal/lysosomal system. It catalyzes the third step of degradation of glucosaminoglycans and is required for the degradation of heparan sulphate. Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A), also known as Sanfilippo syndrome A.

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 :

Glycosaminoglycan Degradation Pathway (82981); Glycosaminoglycan Degradation Pathway (355); Glycosaminoglycan Metabolism Pathway (1269972); HS-GAG Degradation Pathway (1269983); Heparan Sulfate Degradation Pathway (413377); Heparan Sulfate Degradation Pathway (468270); Heparan Sulfate/heparin (HS-GAG) Metabolism Pathway (1269980); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolic Pathways (132956)

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: EC 3.10.1.1; Hydrolase; Glycan Metabolism - glycosaminoglycan degradation. Chromosomal Location of Human Ortholog: 17q25.3. Cellular Component: lysosomal lumen. Molecular Function: catalytic activity; metal ion binding; N-sulfoglucosamine sulfohydrolase activity; sulfuric ester hydrolase activity. Biological Process: carbohydrate metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; proteoglycan metabolic process. Disease: Mucopolysaccharidosis, Type Iiia

size1 :

0.05 mL

price1 :

120 USD

size2 :

0.1 mL

price2 :

160

more info or order :

MyBioSource product webpage