GENESIS OF LYSOSOMAL-ENZYME DEFICIENCIES

[1] SYNTHESIS AND MATURATION OF CROSS-REACTIVE GLYCOPROTEIN IN FIBROBLASTS DEFICIENT IN ARYLSULFATASE A ACTIVITY [J]. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1983, 112 (01) : 198 - 205

[2] AB-VARIANT OF INFANTILE GM2 GANGLIOSIDOSIS - DEFICIENCY OF A FACTOR NECESSARY FOR STIMULATION OF HEXOSAMINIDASE A-CATALYZED DEGRADATION OF GANGLIOSIDE GM2 AND GLYCOLIPID GA2 [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1978, 75 (08) : 3979 - 3983

[3] MOLECULAR DEFECT IN COMBINED BETA-GALACTOSIDASE AND NEURAMINIDASE DEFICIENCY IN MAN [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1982, 79 (15): : 4535 - 4539

[4] IS MULTIPLE SULFATASE DEFICIENCY DUE TO DEFECTIVE REGULATION OF SULFOHYDROLASE EXPRESSION [J]. NATURE, 1979, 282 (5734) : 98 - 100

[5] Gadek JECR, 1983, METABOLIC BASIS INHE

[6] ENZYMATIC PHOSPHORYLATION OF LYSOSOMAL-ENZYMES IN THE PRESENCE OF UDP-N-ACETYLGLUCOSAMINE - ABSENCE OF THE ACTIVITY IN I-CELL FIBROBLASTS [J]. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1981, 98 (03) : 761 - 767

[7] HASILIK A, 1980, J BIOL CHEM, V255, P4937

[8] BIOSYNTHESIS OF LYSOSOMAL-ENZYMES [J]. TRENDS IN BIOCHEMICAL SCIENCES, 1980, 5 (09) : 237 - 240

[9] HASILIK A, 13TH LIND C TRANSL P

[10] ALPHA-GLUCOSIDASE DEFICIENCY IN GENERALIZED GLYCOGEN-STORAGE DISEASE (POMPES DISEASE) [J]. BIOCHEMICAL JOURNAL, 1963, 86 (01) : 11 - &

[1] SYNTHESIS AND MATURATION OF CROSS-REACTIVE GLYCOPROTEIN IN FIBROBLASTS DEFICIENT IN ARYLSULFATASE A ACTIVITY [J]. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1983, 112 (01) : 198 - 205

[2] AB-VARIANT OF INFANTILE GM2 GANGLIOSIDOSIS - DEFICIENCY OF A FACTOR NECESSARY FOR STIMULATION OF HEXOSAMINIDASE A-CATALYZED DEGRADATION OF GANGLIOSIDE GM2 AND GLYCOLIPID GA2 [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1978, 75 (08) : 3979 - 3983

[3] MOLECULAR DEFECT IN COMBINED BETA-GALACTOSIDASE AND NEURAMINIDASE DEFICIENCY IN MAN [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1982, 79 (15): : 4535 - 4539

[4] IS MULTIPLE SULFATASE DEFICIENCY DUE TO DEFECTIVE REGULATION OF SULFOHYDROLASE EXPRESSION [J]. NATURE, 1979, 282 (5734) : 98 - 100

[5] Gadek JECR, 1983, METABOLIC BASIS INHE

[6] ENZYMATIC PHOSPHORYLATION OF LYSOSOMAL-ENZYMES IN THE PRESENCE OF UDP-N-ACETYLGLUCOSAMINE - ABSENCE OF THE ACTIVITY IN I-CELL FIBROBLASTS [J]. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1981, 98 (03) : 761 - 767

[7] HASILIK A, 1980, J BIOL CHEM, V255, P4937

[8] BIOSYNTHESIS OF LYSOSOMAL-ENZYMES [J]. TRENDS IN BIOCHEMICAL SCIENCES, 1980, 5 (09) : 237 - 240

[9] HASILIK A, 13TH LIND C TRANSL P

[10] ALPHA-GLUCOSIDASE DEFICIENCY IN GENERALIZED GLYCOGEN-STORAGE DISEASE (POMPES DISEASE) [J]. BIOCHEMICAL JOURNAL, 1963, 86 (01) : 11 - &