PHOSPHORYLATION OF LYSOSOMAL-ENZYMES IN FIBROBLASTS - MARKED DEFICIENCY OF N-ACETYLGLUCOSAMINE-1-PHOSPHOTRANSFERASE IN FIBROBLASTS OF PATIENTS WITH MUCOLIPIDOSIS-III

被引：20

作者：

WAHEED, A

HASILIK, A

CANTZ, M

VONFIGURA, K

机构：

来源：

HOPPE-SEYLERS ZEITSCHRIFT FUR PHYSIOLOGISCHE CHEMIE | 1982年 / 363卷 / 02期

关键词：

D O I：

10.1515/bchm2.1982.363.1.169

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

引用

页码：169 / 178

页数：10

共 12 条

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[2] DEFICIENT PHOSPHORYLATION OF MANNOSE RESIDUES OF MANNAN IN FIBROBLASTS OF PATIENTS WITH MUCOLIPIDOSE-II AND MUCOLIPIDOSE-III
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[4] ENZYMATIC PHOSPHORYLATION OF LYSOSOMAL-ENZYMES IN THE PRESENCE OF UDP-N-ACETYLGLUCOSAMINE - ABSENCE OF THE ACTIVITY IN I-CELL FIBROBLASTS
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[J]. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1981, 98 (03) : 761 - 767
[5] HASILIK A, 1980, J BIOL CHEM, V255, P4946
[6] HASILIK A, 1980, J BIOL CHEM, V255, P4937
[7] PHOSPHORYLATED OLIGOSACCHARIDES IN LYSOSOMAL-ENZYMES - IDENTIFICATION OF ALPHA-N-ACETYLGLUCOSAMINE (1) PHOSPHO (6) MANNOSE DIESTER GROUPS
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[J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1980, 77 (12): : 7074 - 7078
[8] HASILIK A, 1982, EUR J BIOCH
[9] STUDIES ON URIDINE-DIPHOSPHATE-GLUCOSE
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[10] FIBROBLASTS FROM PATIENTS WITH I-CELL DISEASE AND PSEUDO-HURLER POLYDYSTROPHY ARE DEFICIENT IN URIDINE 5'-DIPHOSPHATE-N-ACETYLGLUCOSAMINE - GLYCOPROTEIN N-ACETYLGLUCOSAMINYLPHOSPHOTRANSFERASE ACTIVITY
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VARKI, A
KORNFELD, S
[J]. JOURNAL OF CLINICAL INVESTIGATION, 1981, 67 (05) : 1574 - 1579

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