Rabbit anti-Human IDUA Polyclonal Antibody | anti-IDUA antibody

IDUA, ID (IDUA, Alpha-L-iduronidase)

Cat. Num. AAA6000318

• Gene Names: IDUA; IDA; MPS1
• Reactivity: Human
• Applications: ELISA, Western Blot, Immunohistochemistry, Flow Cytometry, Functional Assay
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: IDUA; Polyclonal Antibody; ID (IDUA; Alpha-L-iduronidase); Anti -IDUA; anti-IDUA antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Human
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

• Applicable Applications for anti-IDUA antibody: ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC), Flow Cytometry (FC/FACS)
• Application Notes: Suitable for use in Western Blot, Immunohistochemistry, Flow Cytometry, ELISA; Dilution: ELISA: 1:1,000; Western Blot: 1:100-500; Immunohistochemistry: 1:50-100; Flow Cytometry: 1:10-50
• Immunogen: IDUA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 244-274 amino acids from the Central region of human IDUA.
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot analysis in Hela cell line lysates (35ug/lane) using MBS6000318. This demonstrates that MBS6000318 detected the IDUA protein (arrow).)

Testing Data

(All lanes: MBS6000318 (1:2000) Lane 1: human liver lysates Lane 2: human lung lysates Lysates/proteins at 20ug/lane. Secondary goat anti-rabbit IgG, (H+L), Peroxidase conjugated at 1:10,000. Predicted band size: 73kD. Blocking/Dilution buffer: 5% NFDM/TBST.)

Immunohistochemistry (IHC)

(Immunohistochemistry analysis in formalin fixed and paraffin embedded human prostate carcinoma using MBS6000318 followed by peroxidase conjugation of the secondary antibody and DAB staining.)

Related Product Information for anti-IDUA antibody

IDUA 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.

Product Categories/Family for anti-IDUA antibody

Antibodies; Abs to Enzymes

NCBI and Uniprot Product Information

• NCBI GI #: 62089474
• NCBI GeneID: 3425
• UniProt Accession #: P35475
• 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; skeletal morphogenesis; lysosome organization and biogenesis; disaccharide metabolic process; 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