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anti-Idua antibody :: Sheep anti-Human Iduronidase, a-L- Polyclonal Antibody

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Catalog # MBS610786
Unit / Price
  0.1 mg  /  $765 +1 FREE 8GB USB
anti-Idua antibody
Product Name

Iduronidase, a-L- (Idua), Polyclonal Antibody

Also Known As

Iduronidase, a-L- (alpha-L-Iduronidase, IDUA)

Product Synonym Names
Anti -Iduronidase, a-L- (alpha-L-Iduronidase, IDUA)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Chromosome Location
Chromosome: 5; NC_000071.6 (108669106..108684558). Location: 5 E5; 5 53.24 cM
3D Structure
ModBase 3D Structure for P48441
Clonality
Polyclonal
Isotype
IgG
Host
Sheep
Species Reactivity
Human
Specificity
Recognizes human alpha-L-Iduronidase/IDUA.
Purity/Purification
Affinity Purified
Purified by immunoaffinity chromatography.
Form/Format
Supplied as a lyophilized powder from PBS, pH 7.4, 5% trehalose. Reconstitute with 500ul sterile PBS.
Immunogen
Recombinant protein corresponding to aa26-653 from human alpha-L-Iduronidase/IDUA, expressed in NSO cells (AAA81589).
Preparation and Storage
Lyophilized powder may be stored at -20 degree C. Stable for 12 months at -20 degree C. Reconstitute with sterile PBS. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Reconstituted product is stable for 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer. 
Other Notes
Small volumes of anti-Idua antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-Idua antibody
Alpha-L-Iduronidase (IDUA) is a ubiquitously expressed lysosomal glycosyl hydrolase. It hydrolyzes the unsulfated terminal alpha-L-Iduronic acid residues in glycosaminoglycans including dermatan sulfate and heparin sulfate. Mutations in IDUA that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis.
Product Categories/Family for anti-Idua antibody
Applications Tested/Suitable for anti-Idua antibody
ELISA (EL/EIA), Western Blot (WB), Immunoprecipitation (IP)
Application Notes for anti-Idua antibody
Suitable for use in Direct ELISA, Western Blot and Immunoprecipitation.
Dilution: Western Blot: 0.1ug/ml
Immunoprecipitation: 25ug/ml; conditioned cell culture medium spiked with recombinant Human IDUA.
NCBI/Uniprot data below describe general gene information for Idua. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
71,254 Da[Similar Products]
NCBI Official Full Name
iduronidase
NCBI Official Synonym Full Names
iduronidase, alpha-L-
NCBI Official Symbol
NCBI Official Synonym Symbols
6030426D08
  [Similar Products]
NCBI Protein Information
alpha-L-iduronidase
UniProt Protein Name
Alpha-L-iduronidase
Protein Family
UniProt Gene Name
UniProt Entry Name
IDUA_MOUSE
UniProt Comments for Idua
IDUA: Defects in IDUA are the cause of mucopolysaccharidosis type 1H (MPS1H); also known as Hurler syndrome. MPS1H is a severe form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1H usually present, within the first year of life, a combination of hepatosplenomegaly, skeletal deformities, corneal clouding and severe mental retardation. Obstructive airways disease, respiratory infection and cardiac complications usually result in death before 10 years of age. Defects in IDUA are the cause of mucopolysaccharidosis type 1H/S (MPS1H/S); also known as Hurler-Scheie syndrome. MPS1H/S is a form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. MPS1H/S represents an intermediate phenotype of the MPS1 clinical spectrum. It is characterized by relatively little neurological involvement, but most of the somatic symptoms described for severe MPS1 develop in the early to mid-teens, causing considerable loss of mobility. Defects in IDUA are the cause of mucopolysaccharidosis type 1S (MPS1S); also known as Scheie syndrome. MPS1S is a mild form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1S may have little or no neurological involvement, normal stature and life span, but present development of joints stiffness, mild hepatosplenomegaly, aortic valve disease and corneal clouding. Belongs to the glycosyl hydrolase 39 family.

Protein type: Hydrolase; EC 3.2.1.76; Glycan Metabolism - glycosaminoglycan degradation

Cellular Component: lysosome

Molecular Function: L-iduronidase activity; hydrolase activity; hydrolase activity, acting on glycosyl bonds; hydrolase activity, hydrolyzing O-glycosyl compounds

Biological Process: lysosome organization and biogenesis; skeletal morphogenesis; metabolic process; dermatan sulfate catabolic process; carbohydrate metabolic process; cell morphogenesis; limb morphogenesis; chemical homeostasis
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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