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SGSH/Sulphamidase Antibody, Biotin Conjugated

SGSH/Sulphamidase Antibody, Biotin Conjugated size: 0.1ml 333

Price 333
Size 0.1ml
Long name Biotin Conjugated, SGSH/Sulphamidase Polyclonal Antibody
CategoryConjugated Primary Antibodies
ConjugationBiotinylated
Host OrganismRabbit (Oryctolagus cuniculus)
Target AntigenSGSH/Sulphamidase
SpecificityThis is a highly specific antibody against SGSH/Sulphamidase
ModificationUnmodified
Modification siteNone
ClonalityPolyclonal
ClonePolyclonal antibody
Concentration1ug per 1ul
Immunogen_range310-360/502
Subcellular locationLysosome
SourceKLH conjugated synthetic peptide derived from human Sulphamidase
Gene ID number6448
Swiss ProtP51688
Tested applications IHC-P, WB
Recommended dilutions IHC-P(1:100-500), WB(1:100-1000)
Crossreactivity Mouse, Rat, Human
Crossreactive species details not every possible cross-reactivity is known, Due to limited amount of testing and knowledge
Antigen background Defects in the gene encoding sulfamidase are the cause of Sanfilippo syndrome A, Deficiency of lysosomal sulfatases leads to human diseases characterized by the accumulation of either GAGs (glycosaminoglycans) or sulfolipids, It has been suggested that sulfamidase may be involved in the lysosomal degradation of heparan sulfate, MPS3A or SGSH, SFMD, Sanfilippo syndrome A is characterized by severe central nervous system degeneration but relatively mild somatic manifestations, Sulfamidase, also known as HSS, an autosomal recessive lysosomal storage disease caused by impaired degradation of heparan sulfate, is a 502 amino acid lysosome that belongs to the sulfatase family, Sulfatases are enzymes that hydrolyse a diverse range of sulfate esters
Purification methodThis antibody was purified via Protein A
Storage conditions 50% glycerol and 0, Store refrigerated at 2 to 8 degrees Celcius for up to 1 year, 09% sodium azide, Keep the antibody in an aqueous buffered solution containing 1% BSA
Excitation EmissionN/A
Synonyms HSS MPS3A SFMD
Properties Avidin and Streptavidin bind to the small biotin and are couple to HRP or AP for ELISA, Biotin conjugates can be detected by horseradish peroxidase, To break the streptavidin Biotin bond we suggest to use a 6 molar guanidine HCl solution with acidity of pH 1, alkaline phosphatase substrates or anti biotin conjugated antibodies, 6, C, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies they should be stored frozen at - 24°
Gene targetSGSH/Sulphamidase Conjugated
Short name Conjugated, SGSH/Sulphamidase Antibody
Technique antibodies against human proteins, antibodies for, antibody Conjugates, Antibody
LabelBiotin
Alternative name biotinilated coupled, SGSH/Sulphamidase (Antibody to)
Alternative techniqueantibodies
Identity 10818
Gene SGSH
Long gene name N-sulfoglucosamine sulfohydrolase
Synonyms name sulfamidase mucopolysaccharidosis type IIIA
Locus 17q25, 3
Discovery year 1997-06-24
GenBank acession BC047318
Entrez gene record 6448
Pubmed identfication 7493035
RefSeq identity NM_000199
Classification Sulfatases
Havana BLAST/BLAT OTTHUMG00000177569

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