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SGSH/Sulphamidase Polyclonal Antibody, Biotin Conjugated

Applications

  • WB
  • ELISA
  • IHC-P
  • IHC-F

Reactivity

  • Rat

Predicted Reactivity

  • Human
  • Mouse
  • Dog
Overview
Catalog # bs-11756R-Biotin
Product Name SGSH/Sulphamidase Polyclonal Antibody, Biotin Conjugated
Applications WB, ELISA, IHC-P, IHC-F
Reactivity Rat
Predicted Reactivity Human, Mouse, Dog
Specifications
Conjugation Biotin
Host Rabbit
Source KLH conjugated synthetic peptide derived from human Sulphamidase
Immunogen Range 301-388/502
Clonality Polyclonal
Isotype IgG
Concentration 1ug/ul
Purification Purified by Protein A.
Storage Buffer Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage Condition Store at -20°C for 12 months.
Target
Gene ID 6448
Swiss Prot P51688
Subcellular location Cytoplasm
Synonyms HSS; SFMD; MPS3A; N-sulphoglucosamine sulphohydrolase; Sulfoglucosamine sulfamidase; Sulphamidase; SGSH
Background 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.
Application Dilution
WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500