4-Methylumbelliferyl-alpha-N-acetyl-D-galactosaminide

 

Purity:                    Free 4MU < 0.1 %

Biochemical test: Alpha-N-Ac-galactosaminidase activity in fibroblasts, leukocytes and
plasma from Schindler patients (alpha-NAGA deficiency) < 3%,
<12% and < 2 % of mean normal activity, respectively.

Molecular Formula:

C16H19NNaO10S      MW: 439

Literature reference:

Human alpha-N-acetylgalactosaminidase (alpha-NAGA) deficiency:
new mutations and the paradox between genotype and phenotype.
Keulemans JL, Reuser AJ, Kroos MA, Willemsen R, Hermans MM,
van den Ouweland AM, de Jong JG, Wevers RA, Renier WO,
Schindler D, Coll MJ, Chabas A, Sakuraba H, Suzuki Y,
van Diggelen OP.  J Med Genet 1996; 33: 458-464