Human alpha-L-iduronidase (IDUA) ELISA Kit | MyBioSource MBS266949 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human alpha-L-iduronidase (IDUA) ELISA Kit

catalog :

MBS266949

quantity :

48-Strip-Wells

price :

275 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS266949

products type :

ELISA Kit

products full name :

Human alpha-L-iduronidase (IDUA) ELISA Kit

products short name :

alpha-L-iduronidase (IDUA)

products name syn :

Human a-L-iduronidase (IDUA) ELISA Kit

other names :

alpha-L-iduronidase; Alpha-L-iduronidase; alpha-L-iduronidase; iduronidase, alpha-L-

products gene name :

IDUA

other gene names :

IDUA; IDUA; IDA; MPS1

uniprot entry name :

IDUA_HUMAN

reactivity :

Human

sequence length :

653

specificity :

No cross-reaction with other factors.

storage stability :

Store all reagents at 2-8 degree C.

other info1 :

Samples: Human serum, plasma or Cell Culture Supernatant and organizations in the natural and recombinant IDUA concentration. Assay Type: Sandwich. Detection Range: 10 ng/ml-0.156 ng/ml. Sensitivity: 0.05 ng/ml.

other info2 :

Intra-assay Precision: <= 8%. Inter-assay Precision: <= 12%

products description :

Principle of the assay: This experiment use double-sandwich elisa technique and the ELISA Kit provided is typical. The pre-coated antibody is human IDUA monoclonal antibody and the detecting antibody is polyclonal antibody with biotin labeled. Samples and biotin labeling antibody are added into ELISA plate wells and washed out with PBS or TBS. Then Avidin-peroxidase conjugates are added to ELISA wells in order; Use TMB substrate for coloring after reactant thoroughly washed out by PBS or TBS. TMB turns into blue in peroxidase catalytic and finally turns into yellow under the action of acid. The color depth and the testing factors in samples are positively correlated.

ncbi gi num :

110611239

ncbi acc num :

NP_000194.2

ncbi gb acc num :

NM_000203.4

ncbi mol weight :

72,670 Da

ncbi pathways :

CS/DS Degradation Pathway (645311); Chondroitin Sulfate/dermatan Sulfate Metabolism Pathway (645308); Dermatan Sulfate Degradation Pathway (413375); Dermatan Sulfate Degradation Pathway (468240); Disease Pathway (530764); Glycosaminoglycan Degradation Pathway (82981); Glycosaminoglycan Degradation Pathway (355); Glycosaminoglycan Metabolism Pathway (645297); HS-GAG Degradation Pathway (645307); Heparan Sulfate Degradation Pathway (413377)

ncbi summary :

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 summary :

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: EC 3.2.1.76; Hydrolase; Glycan Metabolism - glycosaminoglycan degradation. Chromosomal Location of Human Ortholog: 4p16.3. Cellular Component: lysosomal lumen. Molecular Function: L-iduronidase activity; receptor binding. Biological Process: glycosaminoglycan metabolic process; disaccharide metabolic process; dermatan sulfate catabolic process; cell morphogenesis; pathogenesis; limb morphogenesis; chemical homeostasis; chondroitin sulfate metabolic process; glycosaminoglycan catabolic process; lysosome organization and biogenesis; skeletal morphogenesis; chondroitin sulfate catabolic process; carbohydrate metabolic process. Disease: Hurler-scheie Syndrome; Scheie Syndrome; Hurler Syndrome

size1 :

48-Strip-Wells

price1 :

275 USD

size2 :

96-Strip-Wells

price2 :

420

more info or order :

MyBioSource product webpage