Product Name	SGSH
Chinese Name	磺氨基葡糖硫酸胺酶抗体
Alias	Heparan sulfate sulfatase; Heparan sulphate sulphatase; HSS; MPS 3A; MPS3 A; MPS3A; N sulfoglucosamine sulfohydrolase (sulfamidase); N-sulphoglucosamine sulphohydrolase; SFMD; SGSH; SPHM_HUMAN; Sulfoglucosamine sulfamidase; Sulphamidase; Sulphoglucosamine sulphamidase.
literatures	Specific References  (1)     |     SL11756R has been referenced in 1 publications. [IF=14.404] Wu, Jianjun. et al. ROS-responsive PPGF nanofiber membrane as a drug delivery system for long-term drug release in attenuation of osteoarthritis. NPJ REGEN MED. 2022 Nov;7(1):1-15  IF ;  Rat.   PubMed:36323709
Research Area	Neurobiology  Signal transduction  Cytoskeleton  Extracellular matrix
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Human, Mouse, Rat,  (predicted: Dog, )
Applications	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	55kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human Sulphamidase: 301-388/502
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	Sulfatases are enzymes that hydrolyse a diverse range of sulfate esters. Deficiency of lysosomal sulfatases leads to human diseases characterized by the accumulation of either GAGs (glycosaminoglycans) or sulfolipids. Sulfamidase, also known as HSS, SFMD, MPS3A or SGSH, is a 502 amino acid lysosome that belongs to the sulfatase family. It has been suggested that sulfamidase may be involved in the lysosomal degradation of heparan sulfate. Defects in the gene encoding sulfamidase are the cause of Sanfilippo syndrome A, an autosomal recessive lysosomal storage disease caused by impaired degradation of heparan sulfate. Sanfilippo syndrome A is characterized by severe central nervous system degeneration but relatively mild somatic manifestations. Subcellular Location: Lysosome. Post-translational modifications: The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity. DISEASE: Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A) [MIM:252900]; 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. Similarity: Belongs to the sulfatase family. SWISS: P51688 Gene ID: 6448 Database links: Entrez Gene: 6448 Human Omim: 605270 Human SwissProt: P51688 Human Unigene: 31074 Human
Product Picture	Sample: Lane 1: Mouse NIH/3T3 cell lysates Lane 2: Human MCF-7 cell lysates Lane 3: Human 293T cell lysates Lane 4: Human HUVEC cell lysates Lane 5: Human HeLa cell lysates Primary: Anti-SGSH (SL11756R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 55 kDa Observed band size: 60 kDa Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-SGSH Polyclonal Antibody, Unconjugated(SL11756R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining

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