ELISA/assay

Camel N-Acetylgalactosaminidase Alpha ELISA Kit

MBS053019

48-Strip-Wells

470 USD

ELISA Kit

Camel N-Acetylgalactosaminidase Alpha ELISA Kit

[N-Acetylgalactosaminidase Alpha]

[N-acetylgalactosaminidase, alpha-, isoform CRA_a; Alpha-N-acetylgalactosaminidase; alpha-N-acetylgalactosaminidase; alpha-galactosidase B; Acetylgalactosaminidase, alpha-N- (alpha-galactosidase B); N-acetylgalactosaminidase, alpha-; Alpha-galactosidase B]

[NAGalpha]

[NAGA; NAGA; GALB; D22S674]

Camel

411

Store all reagents at 2-8 degree C

Samples: Porcine Body Fluids And Tissue Homogenates. Assay Type: Quantitative Sandwich. Detection Range: 0.25ug/ml-8ug/ml. Sensitivity: 0.1ug/ml.

Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100]. All CV% should be compared by concentration, not compared by OD values.

Background/Introduction: This Quantitative Sandwich ELISA kit is for lab reagent/research use only, not for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determine the level of NAD (hereafter termed "analyte") in undiluted original Porcine body fluids and tissue homogenates. This kit is NOT suitable for assaying non-biological sources of substances.

119580890

EAW60486.1

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

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: Hydrolase; EC 3.2.1.49; Glycan Metabolism - glycosphingolipid biosynthesis - globo series. 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: Kanzaki Disease; Schindler Disease, Type I

48-Strip-Wells

470 USD

96-Strip-Wells

680

5x96-Strip-Wells

3100

10x96-Strip-Wells

6095