• Call +1.858.633.0165 or Fax +1.858.633.0166 or Contact Us

anti-IDUA antibody :: Rabbit iduronidase, alpha-L- Polyclonal Antibody

Scan QR to view Datasheet
Catalog # MBS710652
Unit / Price
  0.05 mL  /  $230 +1 FREE 8GB USB
  0.15 mL  /  $500 +1 FREE 8GB USB
anti-IDUA antibody
Product Name

iduronidase, alpha-L- (IDUA), Polyclonal Antibody

Popular Item
Also Known As

Rabbit anti-human iduronidase, alpha-L- polyclonal Antibody

Product Synonym Names
iduronidase; alpha-L-; IDUA; IDA; MPS1
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
MBS710652 Technical Datasheet
Immunogen Sequence Length
376
OMIM
607016
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Antigen Affinity Purified
Immunogen
Human IDUA
Storage Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20 degree C, Avoid freeze / thaw cycles.
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-241187 / sc-241189
ISO Certification
Manufactured in an ISO 9001:2008 Certified Laboratory.
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.
Applications Tested/Suitable for anti-IDUA antibody
ELISA (EIA), Western Blot (WB)
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 Secondary Accession #
UniProt Related Accession #
Molecular Weight
73,455 Da[Similar Products]
NCBI Official Full Name
iduronidase, alpha-L-, isoform CRA_j
NCBI Official Synonym Full Names
iduronidase, alpha-L-
NCBI Official Symbol
NCBI Official Synonym Symbols
IDA; MPS1
  [Similar Products]
NCBI Protein Information
alpha-L-iduronidase
UniProt Protein Name
Alpha-L-iduronidase
Protein Family
UniProt Gene Name
UniProt Entry Name
IDUA_HUMAN
NCBI Summary for IDUA
This gene encodes an enzyme that hydrolyzes the terminal alpha-L-iduronic acid residues of two glycosaminoglycans, dermatan sulfate and heparan sulfate. This hydrolysis is required for the lysosomal degradation of these glycosaminoglycans. Mutations in this gene that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I). [provided by RefSeq, Jul 2008]
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: Glycan Metabolism - glycosaminoglycan degradation; EC 3.2.1.76; Hydrolase

Chromosomal Location of Human Ortholog: 4p16.3

Cellular Component: lysosomal lumen

Molecular Function: L-iduronidase activity; receptor binding

Biological Process: glycosaminoglycan metabolic process; disaccharide metabolic process; dermatan sulfate catabolic process; cell morphogenesis; pathogenesis; limb morphogenesis; chemical homeostasis; chondroitin sulfate metabolic process; glycosaminoglycan catabolic process; lysosome organization and biogenesis; skeletal morphogenesis; chondroitin sulfate catabolic process; carbohydrate metabolic process

Disease: Hurler-scheie Syndrome; Scheie Syndrome; Hurler Syndrome
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.
Products associated withanti-IDUA antibody
Request a Quote

Please fill out the form below and our representative will get back to you shortly.

MBS000000
Contact Us

Please fill out the form below and our representative will get back to you shortly.

MBS000000