Human a L iduronidase ELISA Kit | IDUA elisa kit

Human a L iduronidase ELISA Kit

Cat. Num. AAA731526

• Gene Names: IDUA; IDA; MPS1
• Reactivity: Human
• Synonyms: a L iduronidase; Human a L iduronidase ELISA Kit; IDUA elisa kit

• Reactivity: Human

• Samples: Cell culture fluid & body fluid & tissue homogenate Serum or blood plasma
• Preparation and Storage: Store all reagents at 2-8 degree C.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for IDUA elisa kit

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. 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 binding site. 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.

Product Categories/Family for IDUA elisa kit

Signal Transduction

NCBI and Uniprot Product Information

• NCBI GI #: 62089474
• NCBI GeneID: 3425
• Molecular Weight: 72,670 Da
• NCBI Official Full Name: IDUA protein, partial
• NCBI Official Synonym Full Names: iduronidase, alpha-L-
• NCBI Official Symbol: IDUA
• NCBI Official Synonym Symbols: IDA; MPS1
• NCBI Protein Information: alpha-L-iduronidase
• UniProt Protein Name: Alpha-L-iduronidase
• Protein Family: Alpha-L-iduronidase
• UniProt Gene Name: IDUA
• UniProt Entry Name: IDUA_HUMAN

NCBI Description

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 Description

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