IDUA Polyclonal antibody

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  WB analysis using 30006-1-AP

  Various lysates were subjected to SDS PAGE followed by western blot with 30006-1-AP (IDUA antibody) at dilution of 1:1500 incubated at room temperature for 1.5 hours.

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  IHC staining of mouse kidney using 30006-1-AP

  Immunohistochemical analysis of paraffin-embedded mouse kidney tissue slide using 30006-1-AP (IDUA antibody) at dilution of 1:200 (under 40x lens). Heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0).

IDUA Polyclonal Antibody for WB, IHC, ELISA

Cat No. 30006-1-AP

Host / Isotype

Rabbit / IgG

Reactivity

Human, Mouse, rat

Applications

WB, IHC, ELISA

Synonyms

Alpha L iduronidase, IDA, IDUA, iduronidase, alpha L, MPS1

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Tested Applications

• Positive WB detected in: A549 cells, HEK-293 cells, LNCaP cells, mouse brain tissue, rat brain tissue
• Positive IHC detected in: mouse kidney tissue; Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0

Recommended dilution

• Western Blot (WB): WB : 1:500-1:3000
• Immunohistochemistry (IHC): IHC : 1:50-1:500

It is recommended that this reagent should be titrated in each testing system to obtain optimal results.

Sample-dependent, Check data in validation data gallery.

Product Information

30006-1-AP targets IDUA in WB, IHC, ELISA applications and shows reactivity with Human, Mouse, rat samples.

• Tested Reactivity: Human, Mouse, rat
• Host / Isotype: Rabbit / IgG
• Class: Polyclonal
• Type: Antibody
• Immunogen:

  CatNo: Ag30658

  Product name: Recombinant human IDUA protein

  Source: e coli.-derived, PGEX-4T

  Tag: GST

  Domain: 441-555 aa of NM_000203

  Sequence: YASDDTRAHPNRSVAVTLRLRGVPPGPGLVYVTRYLDNGLCSPDGEWRRLGRPVFPTAEQFRRMRAAEDPVAAAPRPLPAGGRLTLRPALRLPSLLLVHVCARPEKPPGQVTRLR

• Full Name: iduronidase, alpha-L-
• Calculated Molecular Weight: 73 kDa
• Observed Molecular Weight: 73 kDa
• GenBank Accession Number: NM_000203
• Gene Symbol: IDUA
• Gene ID (NCBI): 3425
• RRID: AB_2935498
• Conjugate: Unconjugated
• Form: Liquid
• Purification Method: Antigen affinity purification
• UNIPROT ID: P35475
• Storage Buffer: PBS with 0.02% sodium azide and 50% glycerol, pH 7.3.
• Storage Conditions: Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20^oC storage. 20ul sizes contain 0.1% BSA.

Background Information

Iduronidase (L-iduronidase, alpha-L-iduronidase, laronidase) is an enzyme with the systematic name glycosaminoglycan alpha-L-iduronohydrolase. This enzyme catalyzes the hydrolysis of unsulfated alpha-L-iduronosidic linkages in dermatan sulfate. It is a glycoprotein enzyme found in the lysosomes of cells. It is involved in the degeneration of glycosaminoglycans such as dermatan sulfate and heparan sulfate. The enzyme acts by hydrolyzing the terminal alpha-L-iduronic acid residues of these molecules, degrading them (PMID: 4993544,30407). A deficiency in the IDUA protein is associated with mucopolysaccharidoses (MPS). MPS, a type of lysosomal storage disease, is typed I through VII. In this syndrome, glycosaminoglycans accumulate in the lysosomes and cause substantial disease in many different tissues of the body. IDUA mutations result in the MPS 1 phenotype, which is inherited in an autosomal recessive fashion. The defective alpha-L-iduronidase results in an accumulation of heparan and dermatan sulfate within phagocytes, endothelium, smooth muscle cells, neurons, and fibroblasts. Prenatal diagnosis of this enzyme deficiency is possible (PMID:8242073).