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Anti-SGSH Antibody

品牌：Antibodies

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規(guī)格：50μl

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Anti-SGSH Antibody

商品詳情參考文獻(xiàn) 相關(guān)資料

Overview

Name:	Anti-SGSH Antibody See all SGSH primary antibodies
Description:	Rabbit polyclonal antibody to SGSH.
Applications:	WB, IHC
Dilutions:	WB: 1:500 - 1:2000, IHC: 1:50 - 1:100.
Reactivity:	Human
Immunogen:	Recombinant protein of human SGSH.
Protein Length:	502
Host:	Rabbit
Clonality:	Polyclonal
Isotype:	IgG
Conjugate:	Unconjugated
Purification:	Affinity purification.
Product Form:	Liquid
Formulation:	Supplied in Phosphate Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 50% Glycerol.
Storage:	Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

Target

Function:	Catalyzes a step in lysosomal heparan sulfate degradation.
Involvement in Disease:	Mucopolysaccharidosis 3A: 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.
Sequence Similarities:	Belongs to the sulfatase family.
Post-Translational Modification:	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.
Cellular Location:	Lysosome.
Database Links:	Entrez Gene: 6448?Human Omim: 605270?Human SwissProt: P51688?Human Unigene: 31074?Human
Synonyms:	Heparan sulfate sulfatase Antibody Heparan sulphate sulphatase Antibody HSS Antibody MPS 3A Antibody MPS3 A Antibody MPS3A Antibody Mucopolysaccharidosis type IIIA Antibody N sulfoglucosamine sulfohydrolase Antibody N sulfoglucosamine sulfohydrolase (sulfamidase) Antibody N-sulphoglucosamine sulphohydrolase Antibody SFMD Antibody SGSH Antibody SPHM_HUMAN Antibody Sulfoglucosamine sulfamidase Antibody Sulphamidase Antibody Sulphoglucosamine sulphamidase Antibody
Information:	Target information shown above is from the UniProt Consortium.