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

Scan QR to view Datasheet Catalog #    MBS610786 anti-Idua antibody
Unit / Price
0.1 mg  /  $755 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 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)
 Product Gene Name   

anti-Idua antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Chromosome Location    Chromosome: 5; NC_000071.6 (108669106..108684558). Location: 5 E5; 5 53.24 cM
 3D Structure    ModBase 3D Structure for P48441
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Sheep
 Species Reactivity    Human
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 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.
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 Immunogen    Recombinant protein corresponding to aa26-653 from human alpha-L-Iduronidase/IDUA, expressed in NSO cells (AAA81589).
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 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.
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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    Antibodies; Abs to Enzymes
 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.
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NCBI/Uniprot data below describe general gene information for Idua. It may not necessarily be applicable to this product.
 NCBI GI #    623170
 NCBI GeneID    15932
 NCBI Accession #    AAC42044.1 [Other Products]
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 UniProt Primary Accession #    P48441 [Other Products]
 UniProt Secondary Accession #    E9QM17 [Other Products]
 UniProt Related Accession #    P48441 [Other Products]
 Molecular Weight    71,254 Da [Similar Products]
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 NCBI Official Full Name    iduronidase
 NCBI Official Synonym Full Names    iduronidase, alpha-L-
 NCBI Official Symbol    Idua [Similar Products]
 NCBI Official Synonym Symbols   
6030426D08
[Similar Products]
 NCBI Protein Information    alpha-L-iduronidase
 UniProt Protein Name    Alpha-L-iduronidase
 Protein Family    Alpha-L-iduronidase
 UniProt Gene Name    Idua [Similar Products]
 UniProt Entry Name    IDUA_MOUSE
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 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
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 Research Articles on Idua    1. Mucopolysaccharidosis type I, unique structure of accumulated heparan sulfate and increased N-sulfotransferase activity in mice lacking alpha-l-iduronidase.
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 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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Pathways associated with anti-Idua antibodyOrgans/Tissues associated with anti-Idua antibody
 Products by Pathway  Pathway Diagram
 CS/DS Degradation Pathway antibodies  CS/DS Degradation Pathway Diagram
 Chondroitin Sulfate/dermatan Sulfate Metabolism Pathway antibodies  Chondroitin Sulfate/dermatan Sulfate Metabolism Pathway Diagram
 Dermatan Sulfate Degradation Pathway antibodies  Dermatan Sulfate Degradation Pathway Diagram
 Dermatan Sulfate Degradation Pathway antibodies  Dermatan Sulfate Degradation Pathway Diagram
 Disease Pathway antibodies  Disease Pathway Diagram
 Glycosaminoglycan Degradation Pathway antibodies  Glycosaminoglycan Degradation Pathway Diagram
 Glycosaminoglycan Degradation Pathway antibodies  Glycosaminoglycan Degradation Pathway Diagram
 Glycosaminoglycan Metabolism Pathway antibodies  Glycosaminoglycan Metabolism Pathway Diagram
 HS-GAG Degradation Pathway antibodies  HS-GAG Degradation Pathway Diagram
 Heparan Sulfate Degradation Pathway antibodies  Heparan Sulfate Degradation Pathway Diagram
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >48 publications with Idua and Blood
 Brain Antibodies  >43 publications with Idua and Brain
 Liver Antibodies  >28 publications with Idua and Liver
 Spleen Antibodies  >15 publications with Idua and Spleen
 Kidney Antibodies  >11 publications with Idua and Kidney
 Skin Antibodies  >9 publications with Idua and Skin
 Lung Antibodies  >9 publications with Idua and Lung
 Ovary Antibodies  >7 publications with Idua and Ovary
 Eye Antibodies  >5 publications with Idua and Eye
 Spinal Cord Antibodies  >4 publications with Idua and Spinal Cord
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