Is there a mechanism for introducing acid hydrolases into liver lysosomes that is independent of mannose 6-phosphate recognition? evidence from I-cell disease
- 1 April 1982
- journal article
- research article
- Published by Elsevier in Biochemical and Biophysical Research Communications
- Vol. 105 (3), 814-820
- https://doi.org/10.1016/0006-291x(82)91042-7
Abstract
No abstract availableThis publication has 22 references indexed in Scilit:
- Defective phosphorylation and processing of β-hexosaminidase by intact cultured fibroblasts from patients with mucolipidosis IIIArchives of Biochemistry and Biophysics, 1982
- Fibroblasts from patients with I-cell disease and pseudo-Hurler polydystrophy are deficient in uridine 5'-diphosphate-N-acetylglucosamine: glycoprotein N-acetylglucosaminylphosphotransferase activity.Journal of Clinical Investigation, 1981
- Enzymatic phosphorylation of lysosomal enzymes in the presence of UDP-N-acetylglucosamine. Absence of the activity in l-cell fibroblastsBiochemical and Biophysical Research Communications, 1981
- I-cell disease: Deficiency of extracellular hydrolase phosphorylationBiochemical and Biophysical Research Communications, 1979
- Two species of lysosomal organelles in cultured human fibroblastsCell, 1979
- Isoelectric focusing of acid hydrolases in human liver and serum. Findings in sera from one patient with I-cell disease phenotypeClinica Chimica Acta; International Journal of Clinical Chemistry, 1978
- Inherited Disorders of Lysosomal MetabolismAnnual Review of Biochemistry, 1975
- I-Cell disease (Mucolipidosis II)Acta Neuropathologica, 1975
- Hepatic Ultrastructure and Histochemistry in Mucolipidosis II (I-Cell Disease)Pediatric Research, 1973
- Clinical, biochemical, and ultrastructural studies in a case of chondrodystrophy presenting the I-cell phenotype in tissue cultureThe Journal of Pediatrics, 1971