Mouse IDUA ELISA Kit | IDUA elisa kit

Mouse IDUA (Alpha-L-iduronidase) ELISA Kit

Cat. Num. AAA2507407

• Gene Names: IDUA; IDA; MPS1
• Reactivity: Mouse
• Synonyms: IDUA; Mouse IDUA (Alpha-L-iduronidase) ELISA Kit; IDUA elisa kit

• Reactivity: Mouse
• Specificity: This kit recognizes natural and recombinantMouseIDUA. No significant cross-reactivity or interference between MouseIDUA and analogues was observed.
• Sequence Length: 523

• Samples: Serum, Plasma, Biological Fluids
• Assay Type: Sandwich
• Detection Range: 0.156-10ng/mL
• Sensitivity: Min: 0.094ng/mL; Max: 10ng/mL
• Preparation and Storage: Store at 4 degree C.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for IDUA elisa kit

Intended Uses: This ELISA kit applies to the invitro quantitative determination of MouseIDUAconcentrations in serum, plasma and other biological fluids.

Principle of the Assay: This ELISA kit uses Sandwich-ELISA as the method. The micro ELISA plate provided in this kit has been pre-coated with an antibody specific to IDUA. Standards or samples are added to the appropriate micro ELISA plate wells and combined with the specific antibody. Then a biotinylated detection antibody specific for IDUAand Avidin-Horseradish Peroxidase (HRP) conjugate is added to each micro plate well successively and incubated. Free components are washed away. The substrate solution is added to each well. Only those wells that contain IDUA, biotinylated detection antibody and Avidin-HRP conjugate will appear blue in color. The enzyme-substrate reaction is terminated by the addition of a sulphuric acid solution and the color turns yellow. The optical density (OD) is measured spectrophotometrically at a wavelength of 450 nm +/- 2 nm. The OD value is proportional to the concentration of IDUA.You can calculate the concentration of IDUAin the samples by comparing the OD of the samples to the standard curve.

NCBI and Uniprot Product Information

• NCBI GI #: 62089474
• NCBI GeneID: 3425
• UniProt Accession #: P35475; B3KWK6
• Molecular Weight: 73,455 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; disaccharide metabolic process; skeletal morphogenesis; lysosome organization and biogenesis; dermatan sulfate catabolic process; carbohydrate metabolic process; chondroitin sulfate catabolic process; cell morphogenesis; pathogenesis; limb morphogenesis; chemical homeostasis

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