SGSH Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IHC, WB
Recommend Dilution	IHC:1:100-1:1000; WB:1:1000-1:10000
Reactivity	Human, Mouse, Cow, Dog
Host	Rabbit
Clonality	Polyclonal
Immunogen	Recombinant fragment corresponding to a region within amino acids 318 and 496 of SGSH (Uniprot ID#P51688)
Formulation	0.1M Tris, 0.1M Glycine, 10% Glycerol (pH7). 0.01% Thimerosal was added as a preservative.
Concentration	lot specific
Purification	Purified by antigen-affinity chromatography.
Conjugation	Unconjugated
Storage Condition	Store at -20°C as received.
Predicted Protein Size	57 kDa
Gene Name	N-sulfoglucosamine sulfohydrolase
Database Link	NP_000190 Entrez Gene 27029 MouseEntrez Gene 403707 DogEntrez Gene 6448 Human UniProt ID P51688
Background	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]
Synonyms	HSS; MPS3A; SFMD
Note	Seq homology of immunogen across species: mouse (81%), cow (81%), Dog (82%)
Reference Data
Protein Families	Druggable Genome
Protein Pathways	Glycosaminoglycan degradation, Lysosome, Metabolic pathways

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