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

IDUA Antibody (Center)

Cat. Num. AAA9214679

• Gene Names: IDUA; IDA; MPS1
• Reactivity: Human
• Applications: Western Blot, ELISA, Immunohistochemistry, Flow Cytometry, Functional Assay
• Purity: Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
• Synonyms: IDUA; Polyclonal Antibody; IDUA Antibody (Center); Alpha-L-iduronidase; anti-IDUA antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit Ig
• Specificity: This IDUA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 236-264 amino acids from the Central region of human IDUA.
• Purity/Purification: Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
• Form/Format: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
• Concentration: Vial Concentration: 0.25 (varies by lot)
• Sequence Positions: 236-264
• Sequence Length: 675

• Applicable Applications for anti-IDUA antibody: Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC), Flow Cytometry (FC/FACS)
• Application Notes: WB~~1:1000
• Antigen Type: Synthetic Peptide
• Antigen Source: HUMAN
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Western Blot (WB)

(Anti-IDUA Antibody (Center)at 1:2000 dilution + human liver lysatesLysates/proteins at 20 ug per lane.SecondaryGoat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilutionPredicted band size : 73 kDaBlocking/Dilution buffer: 5% NFDM/TBST.)

Western Blot (WB)

(Western blot analysis of lysates from A549 cell line, rat lung tissue lysate (from left to right), using IDUA Antibody (Center). MBS9214679 was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L(HRP) at 1:10000 dilution was used as the secondary antibody. Lysates at 35ug per lane.)

Western Blot (WB)

(IDUA Antibody (Center) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the IDUA antibody detected the IDUA protein (arrow).)

Immunohistochemistry (IHC)

(IDUA Antibody (Center) immunohistochemistry analysis in formalin fixed and paraffin embedded human prostate carcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the IDUA Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.)

Flow Cytometry (FC/FACS)

(IDUA Antibody (Center) flow cytometric analysis of Hela cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

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

Metabolism; Signal Transduction

References

Amr, K., et al. Genet Test Mol Biomarkers 13(6):761-764(2009)
Vazna, A., et al. Am. J. Med. Genet. A 149A (5), 965-974 (2009)
Sugawara, K., et al. J. Hum. Genet. 53(5):467-474(2008)

NCBI and Uniprot Product Information

• NCBI GI #: 110611239
• NCBI GeneID: 3425
• NCBI Accession #: NP_000194.2
• NCBI GenBank Nucleotide #: NM_000203.4
• UniProt Accession #: P35475; B3KWK6
• Molecular Weight: 72670
• NCBI Official Full Name: alpha-L-iduronidase
• 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: Hydrolase; Glycan Metabolism - glycosaminoglycan degradation; 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; skeletal morphogenesis; disaccharide metabolic process; 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