Anti-Iduronate 2 sulfatase antibody | MyBioSource MBS176148 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Anti-Iduronate 2 sulfatase antibody

catalog :

MBS176148

quantity :

0.1 mg

price :

245 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human

application :

western blot, immunohistochemistry, immunohistochemistry - paraffin section

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS176148

products type :

Antibody

products full name :

Anti-Iduronate 2 sulfatase antibody

products short name :

Iduronate 2 sulfatase

products name syn :

Iduronate 2-sulfatase; iduronate 2-sulfatase; Alpha L iduronate sulfate sulfatase antibody; Alpha-L-iduronate sulfate sulfatase antibody; AW214631 antibody; Ids antibody; IDS_HUMAN antibody; Iduronate 2 sulfatase 14 kDa chain antibody; Iduronate 2 sulfatase 42 kDa chain antibody; Iduronate 2 sulfatase antibody; Iduronate 2-sulfatase 14 kDa chain antibody; Iduronate sulfatase antibody; Idursulfase antibody; MPS2 antibody; RP23-29M4.1 antibody; SIDS antibody

other names :

Iduronate 2-sulfatase; Iduronate 2-sulfatase; iduronate 2-sulfatase; idursulfase; iduronate 2-sulfatase 14 kDa chain; iduronate 2-sulfatase 42 kDa chain; alpha-L-iduronate sulfate sulfatase; iduronate 2-sulfatase; Alpha-L-iduronate sulfate sulfatase; Idursulfase

products gene name :

IDS

other gene names :

IDS; IDS; MPS2; SIDS; SIDS; Idursulfase

uniprot entry name :

IDS_HUMAN

clonality :

Polyclonal

isotype :

IgG

host :

Rabbit

reactivity :

Human

sequence length :

550

purity :

Immunogen affinity purified.

form :

Lyophilized

storage stability :

At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

tested application :

Western Blot (WB), Immunohistochemistry (IHC) Paraffin

other info1 :

Immunogen: A synthetic peptide corresponding to a sequence at the C-terminus of human Iduronate 2 sulfatase (430-448aa ELCREGKNLLKHFRFRDLE).

other info2 :

Contents: Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

products description :

Description: Rabbit IgG polyclonal antibody for Iduronate 2-sulfatase(IDS) detection. Tested with WB, IHC-P in Human. Background: IDS(Iduronate-2-sulfatase) is a sulfatase enzyme associated with Hunter syndrome. Iduronate 2-sulfatase is involved in the lysosomal degradation of the glycosaminoglycans heparan sulfate and dermatan sulfate. Wilson et al.(1991) used an IDS cDNA clone to localize the gene to Xq28, distal to the fragile X site. Faust et al.(1992) and Daniele et al.(1993) demonstrated that the homologous Ids gene in the mouse occupies the same position on the X chromosome in relation to the FMR1, F9, and GABRA3 genes. Iduronate-2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this X-chromosome gene that result in enzymatic deficiency lead to the sex-linked mucopolysaccharidosis type II, also known as Hunter syndrome. Iduronate-2-sulfatase has a strong sequence homology with human arylsulfatases A, B, and C, and human glucosamine-6-sulfatase.

ncbi gi num :

124174

ncbi acc num :

P22304.1

uniprot acc num :

P22304

ncbi mol weight :

34,893 Da

ncbi pathways :

CS/DS Degradation Pathway (645311); Chondroitin Sulfate/dermatan Sulfate Metabolism Pathway (645308); Dermatan Sulfate Degradation Pathway (413375); Dermatan Sulfate Degradation Pathway (468240); Disease Pathway (530764); Glycogen Storage Diseases Pathway (980468); Glycosaminoglycan Degradation Pathway (82981); Glycosaminoglycan Degradation Pathway (355); Glycosaminoglycan Metabolism Pathway (645297); HS-GAG Degradation Pathway (645307)

ncbi summary :

The protein encoded by this gene belongs to the sulfatase family, is localized to the lysosome, and is involved in lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this gene are associated with the X-linked lysosomal storage disease, mucopolysaccharidosis type II, also known as Hunter syndrome. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Aug 2013]

uniprot summary :

IDS: Required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Defects in IDS are the cause of mucopolysaccharidosis type 2 (MPS2); also known as Hunter syndrome. MPS2 is an X-linked lysosomal storage disease characterized by intracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine. Most children with MPS2 have a severe form with early somatic abnormalities including skeletal deformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominent feature is neurological damage that presents as developmental delay and hyperactivity but progresses to mental retardation and dementia. They die before 15 years of age, usually as a result of obstructive airway disease or cardiac failure. In contrast, those with a mild form of MPS2 may survive into adulthood, with attenuated somatic complications and often without mental retardation. Belongs to the sulfatase family. 3 isoforms of the human protein are produced by alternative splicing. Protein type: Hydrolase; EC 3.1.6.13; Glycan Metabolism - glycosaminoglycan degradation. Chromosomal Location of Human Ortholog: Xq28. Cellular Component: lysosomal lumen. Molecular Function: metal ion binding; iduronate-2-sulfatase activity. Biological Process: chondroitin sulfate metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; chondroitin sulfate catabolic process; pathogenesis. Disease: Mucopolysaccharidosis, Type Ii

size1 :

0.1 mg

price1 :

245 USD

more info or order :

MyBioSource product webpage