Monkey a-L-iduronidase ELISA Kit | IDUA elisa kit

Monkey a-L-iduronidase ELISA Kit

Cat. Num. AAA744839

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

• Reactivity: Monkey

• Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate
• Sensitivity: 0.1 ng/mL.
• Intended Uses: This IDUA ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Monkey IDUA. This ELISA kit for research use only, not for therapeutic applications!
• Preparation and Storage: Store all reagents at 2-8 degree C.

Typical Testing Data/Standard Curve (for reference only)

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; Hydrolase; EC 3.2.1.76

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; carbohydrate metabolic process; chondroitin sulfate catabolic process; cell morphogenesis; pathogenesis; limb morphogenesis; chemical homeostasis

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