SGSH is 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.
Applications:Suitable for use in ELISA and Western Blot.
Other applications not tested.
Recommended Dilutions:Western Blot: 1:4000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size:200ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by Protein A and peptide affinity chromatography.
Form:Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.
Specificity:Recognizes human SGSH.
Isotype:IgG
Calc Applications Abbrev:E WB
Calc Crossreactivity:Hu
Immunogen:KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 420-449 in the C-terminal of human SGSH. Cellular Localization: Lysosome.