Human Alpha-N-acetylgalactosaminidase (NAGA) ELISA kit; D22S674; GALB; Acetylgalactosaminidase; alpha-N- (alpha-galactosidase B) ; OTTHUMP00000199655; OTTHUMP00000199657; alpha-N-acetylgalactosaminidase; N-acetylgalactosaminidase; alpha-

For Research Use Only. Not for use in diagnostic procedures.

This assay has high sensitivity and excellent specificity for detection of human NAGA. No significant cross-reactivity or interference between human NAGA and analogues was observed.

Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of NAGA elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS937990 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the N-acetylgalactosaminidase, alpha- (NAGA) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing NAGA. The ELISA analytical biochemical technique of the MBS937990 kit is based on NAGA antibody-NAGA antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect NAGA antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, NAGA. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Principle of the Assay: This assay employs the quantitative sandwich enzyme immunoassay technique. Antibody specific for NAGA has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any NAGA present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for NAGA is added to the wells. After washing, avidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of NAGA bound in the initial step. The color development is stopped and the intensity of the color is measured.

46,565 Da

N-acetylgalactosaminidase, alpha-

alpha-N-acetylgalactosaminidase; alpha-galactosidase B; Acetylgalactosaminidase, alpha-N- (alpha-galactosidase B)

Alpha-N-acetylgalactosaminidase

NAGAB_HUMAN

NAGA encodes the lysosomal enzyme alpha-N-acetylgalactosaminidase, which cleaves alpha-N-acetylgalactosaminyl moieties from glycoconjugates. Mutations in NAGA have been identified as the cause of Schindler disease types I and II (type II also known as Kanzaki disease). [provided by RefSeq, Jul 2008]

NAGA: Removes terminal alpha-N-acetylgalactosamine residues from glycolipids and glycopeptides. Required for the breakdown of glycolipids. Defects in NAGA are the cause of Schindler disease (SCHIND). Schindler disease is a form of NAGA deficiency characterized by early onset neuroaxonal dystrophy and neurological signs (convulsion during fever, epilepsy, psychomotor retardation and hypotonia). NAGA deficiency is typically classified in three main phenotypes: NAGA deficiency type I (Schindler disease or Schindler disease type I) with severe manifestations; NAGA deficiency type II (Kanzazi disease or Schindler disease type II) which is mild; NAGA deficiency type III (Schindler disease type III) characterized by mild-to-moderate neurologic manifestations. NAGA deficiency results in the increased urinary excretion of glycopeptides and oligosaccharides containing alpha-N-acetylgalactosaminyl moieties. Inheritance is autosomal recessive. Defects in NAGA are the cause of Kanzaki disease (KANZD); also known as NAGA deficiency type II or Schindler disease type II. Kanzaki disease is an autosomal recessive disorder characterized by late onset, angiokeratoma corporis diffusum and mild intellectual impairment. Belongs to the glycosyl hydrolase 27 family.

Protein type: Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Hydrolase; EC 3.2.1.49

Chromosomal Location of Human Ortholog: 22q11

Cellular Component: lysosome; cytoplasm

Molecular Function: protein homodimerization activity; alpha-galactosidase activity; alpha-N-acetylgalactosaminidase activity

Biological Process: glycolipid catabolic process; carbohydrate catabolic process; glycoside catabolic process; glycosylceramide catabolic process; oligosaccharide metabolic process

Disease: Schindler Disease, Type I; Kanzaki Disease

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