Human Alpha-N-acetylgalactosaminidase, NAGA ELISA Kit | MyBioSource MBS937990 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human Alpha-N-acetylgalactosaminidase, NAGA ELISA Kit

catalog :

MBS937990

quantity :

48-Strip-Wells

price :

510 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS937990

products type :

ELISA Kit

products full name :

Human Alpha-N-acetylgalactosaminidase, NAGA ELISA Kit

products short name :

N-acetylgalactosaminidase, alpha-

products name syn :

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

other names :

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

products gene name :

NAGA

other gene names :

NAGA; NAGA; GALB; D22S674

uniprot entry name :

NAGAB_HUMAN

reactivity :

Human

sequence length :

411

specificity :

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.

storage stability :

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

other info1 :

Samples: Serum, plasma, urine, saliva, tissue homogenates. Assay Type: Sandwich. Detection Range: 0.625 ng/ml-40 ng/ml. Sensitivity: 0.156 ng/ml

other info2 :

Intra-assay Precision: Intra-assay Precision (Precision within an assay): CV%<8%. Three samples of known concentration were tested twenty times on one plate to assess. Inter-assay Precision: Inter-assay Precision (Precision between assays): CV%<10%. Three samples of known concentration were tested in twenty assays to assess.

products description :

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.

ncbi gi num :

4557781

ncbi acc num :

NP_000253.1

ncbi gb acc num :

NM_000262.2

uniprot acc num :

P17050

ncbi mol weight :

46,565 Da

ncbi pathways :

Glycosphingolipid Biosynthesis - Globo Series Pathway (82996); Glycosphingolipid Biosynthesis - Globo Series Pathway (371); Lysosome Pathway (99052); Lysosome Pathway (96865)

ncbi summary :

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]

uniprot summary :

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

size1 :

48-Strip-Wells

price1 :

510 USD

size2 :

96-Strip-Wells

price2 :

725

size3 :

5x96-Strip-Wells

price3 :

2565

size4 :

10x96-Strip-Wells

price4 :

4800

more info or order :

MyBioSource product webpage