Mouse Alpha-L-iduronidase ELISA Kit | Idua elisa kit

Mouse Alpha-L-iduronidase ELISA Kit

Cat. Num. AAA2881549

• Gene Names: Idua; 6030426D08
• Reactivity: Mouse
• Synonyms: Alpha-L-iduronidase; Mouse Alpha-L-iduronidase ELISA Kit; Idua; 3.2.1.76; Idua elisa kit

• Reactivity: Mouse
• Sequence Length: 634

• Assay Type: Sandwich
• Detection Range: 1.56-100 ng/mL
• Sensitivity: 0.8 ng/mL
• Intra-Assay CV: <=8.3%
• Inter-Assay CV: <=10.4%
• Recovery: 92%
• Preparation and Storage: For long term storage, please store the entire kit at -20 degree C.

Typical Testing Data/Standard Curve (for reference only)

NCBI and Uniprot Product Information

• NCBI GI #: 341940818
• NCBI GeneID: 15932
• UniProt Accession #: P48441; E9QM17
• Molecular Weight: 71,254 Da
• NCBI Official Full Name: Alpha-L-iduronidase
• NCBI Official Synonym Full Names: iduronidase, alpha-L-
• NCBI Official Symbol: Idua
• NCBI Official Synonym Symbols: 6030426D08
• 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_MOUSE

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

Cellular Component: lysosome

Molecular Function: hydrolase activity; hydrolase activity, acting on glycosyl bonds; hydrolase activity, hydrolyzing O-glycosyl compounds; L-iduronidase activity; receptor binding

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