a L iduronidase, ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C.

We suggest pre-experimenting with neat (undiluted) samples, 1:2 or 1:4 dilutions. Please avoid diluting your samples more than 1:10 as it would exceed the dilution limit set for this kit. If the expected concentration of the target is beyond the detection range of the kit, please contact our technical support team

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of IDUA elisa kit 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.

MBS731526 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the a L iduronidase (IDUA) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing IDUA. The ELISA analytical biochemical technique of the MBS731526 kit is based on IDUA antibody-IDUA antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect IDUA antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, IDUA. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

For Samples: Cell culture fluid & body fluid & tissue homogenate Serum or blood plasma

Intended Uses: This IDUA ELISA kit is intended for laboratory research use only and not for use in diagnostic or therapeutic procedures. The stop solution changes the color from blue to yellow and the intensity of the color is measured at 450 nm using a spectrophotometer. In order to measure the concentration of IDUA in the sample, this IDUA ELISA Kit includes a set of calibration standards. The calibration standards are assayed at the same time as the samples and allow the operator to produce a standard curve of Optical Density versus IDUA concentration. The concentration of in the samples is then determined by comparing the O.D. of the samples to the standard curve.

Principle of the Assay: The coated well immunoenzymatic assay for the quantitative measurement of IDUA utilizes a multiclonal anti-IDUA antibody and an IDUA-HRP conjugate. The assay sample and buffer are incubated together with IDUA-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the IDUA concentration since IDUA from samples and IDUA-HRP conjugate compete for the anti-IDUA antibody . Since the number of sites is limited, as more sites are occupied by IDUA from the sample, fewer sites are left to bind IDUA-HRP conjugate. Standards of known IDUA concentrations are run concurrently with the samples being assayed and a standard curve is plotted relating the intensity of the color (Optical Density) to the concentration of IDUA. The IDUA concentration in each sample is interpolated from this standard curve.

Signal Transduction

72,670 Da

iduronidase, alpha-L-

alpha-L-iduronidase

Alpha-L-iduronidase

IDUA_HUMAN

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]

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

Disease: Hurler-scheie Syndrome; Scheie Syndrome; Hurler Syndrome

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