Synthesis of a fluorescent derivative of glucosyl ceramide for the sensitive determination of glucocerebrosidase activity.

Citations & References

  • Authors: Dinur T, Grabowski GA, Desnick RJ, Gatt S
  • Journal: Anal Biochem (1984) 136:223-234
  • ID: PN2981
Catalog #
f7b49030b84b97848504c5f439564b69_Citations

Prenatal diagnosis of Krabbe disease using a fluorescent derivative of galactosylceramide.

Citations & References

  • Authors: Zeigler M, Zlotogora J, Regev R, Dagan A, Gatt S, Bach G
  • Journal: Clin Chim Acta (1984) 142:313-318
  • ID: PN26810
Catalog #
1f79c37c38ef31e2174277b34e5aa64b_Citations

Human acid beta-glucosidase: use of inhibitors, alternative substrates and amphiphiles to investigate the properties of the normal and Gaucher disease active sites.

Citations & References

  • Authors: Osiecki-Newman K, Fabbro D, Legler G, Desnick RJ, Grabowski GA
  • Journal: Biochim Biophys Acta (1987) 915:87-100
  • ID: PN26674
e1f27a3eba8e89570965166a129933ec_Citations

Human acid beta-glucosidase. N-glycosylation site occupancy and the effect of glycosylation on enzymatic activity.

Citations & References

  • Authors: Berg-Fussman A, Grace ME, Ioannou Y, Grabowski GA
  • Journal: J Biol Chem (1993) 268:14861-14866
  • ID: PN26679
Catalog #
39ceea9d66d6fd7959821277b8d0af58_Citations

Abnormal transport along the lysosomal pathway in mucolipidosis, type IV disease.

Citations & References

  • Authors: Chen CS, Bach G, Pagano RE
  • Journal: Proc Natl Acad Sci U S A (1998) 95:6373-6378
  • ID: PN30070
11a0674eabe73a56fb088d127536a7d5_Citations

Membrane traffic in sphingolipid storage diseases.

Citations & References

  • Authors: Pagano RE, Puri V, Dominguez M, Marks DL
  • Journal: Traffic (2000) 1:807-815
  • ID: PN40294
232a03118725240918c5a4a0ab33e9b3_Citations

Gaucher type I (Ashkenazi) disease: a new method for heterozygote detection using a novel fluorescent natural substrate.

Citations & References

  • Authors: Grabowski GA, Dinur T, Gatt S, Desnick RJ
  • Journal: Clin Chim Acta (1982) 124:123-135
  • ID: PN26811
1474b89337c66907c36ed7c6c82c592f_Citations

Metabolism of Glucocerebrosides. II. Evidence of an Enzymatic Deficiency in Gaucher′s Disease.

Citations & References

  • Authors: Brady RO, Kanfer JN, Shapiro D
  • Journal: Biochem Biophys Res Comm (1965) 18:221-221
  • ID: PN26673
1bd69c7df3112fb9a584fbd9edfc6c90_Citations

Molecular basis of GM1 gangliosidosis and Morquio disease, type B. Structure-function studies of lysosomal beta-galactosidase and the non-lysosomal beta-galactosidase-like protein.

Citations & References

  • Authors: Callahan JW
  • Journal: Biochim Biophys Acta (1999) 1455:85-103
  • ID: PN40712
547e254ad67a3f2b4cd98de28e663293_Citations