DEFICIENCY OF THE HEXOSAMINIDASE-A ACTIVATOR PROTEIN IN A CASE OF GM2 GANGLIOSIDOSIS - VARIANT-AB

被引：59

作者：

HECHTMAN, P

GORDON, BA

KIN, NMKNY

机构：

[1] CHILDRENS PSYCHIAT RES INST, BIOCHEM LAB, LONDON, ONTARIO, CANADA

[2] MCGILL UNIV, CTR HUMAN GENET, DEPT BIOL, MONTREAL H3C 3G1, QUEBEC, CANADA

[3] MONTREAL NEUROL INST & HOSP, DONNER LAB NEUROCHEM, MONTREAL H3A 2B4, QUEBEC, CANADA

来源：

PEDIATRIC RESEARCH | 1982年 / 16卷 / 03期

关键词：

D O I：

10.1203/00006450-198203000-00011

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

引用

页码：217 / 222

页数：6

共 38 条

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[J]. ACTA NEUROPATHOLOGICA, 1975, 33 (03) : 207 - 226
[2] NIEMANN-PICK DISEASE, TYPE-C - EVIDENCE FOR THE DEFICIENCY OF AN ACTIVATING FACTOR STIMULATING SPHINGOMYELIN AND GLUCOCEREBROSIDE DEGRADATION
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[J]. HOPPE-SEYLERS ZEITSCHRIFT FUR PHYSIOLOGISCHE CHEMIE, 1980, 361 (10): : 1489 - 1502
[3] AB-VARIANT OF INFANTILE GM2 GANGLIOSIDOSIS - DEFICIENCY OF A FACTOR NECESSARY FOR STIMULATION OF HEXOSAMINIDASE A-CATALYZED DEGRADATION OF GANGLIOSIDE GM2 AND GLYCOLIPID GA2
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SANDHOFF, K
[J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1978, 75 (08) : 3979 - 3983
[4] PURIFICATION, BIOCHEMICAL AND IMMUNOLOGICAL CHARACTERIZATION OF HEXOSAMINIDASE-A FROM VARIANT AB OF INFANTILE GM2 GANGLIOSIDOSIS
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[J]. EUROPEAN JOURNAL OF BIOCHEMISTRY, 1978, 84 (01): : 27 - 33
[5] CONZELMANN E, 1980, STRUCTURE FUNCTION G, P295
[6] DISC ELECTROPHORESIS .2. METHOD AND APPLICATION TO HUMAN SERUM PROTEINS
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[J]. ANNALS OF THE NEW YORK ACADEMY OF SCIENCES, 1964, 121 (A2) : 404 - &
[7] ACTIVATOR OF CEREBROSIDE SULFATASE - PURIFICATION FROM HUMAN LIVER AND IDENTIFICATION AS A PROTEIN
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[J]. HOPPE-SEYLERS ZEITSCHRIFT FUR PHYSIOLOGISCHE CHEMIE, 1975, 356 (05): : 605 - 613
[8] FOLCH J, 1957, J BIOL CHEM, V226, P497
[9] THE AB-VARIANT OF GM2-GANGLIOSIDOSIS - CLINICAL, BIOCHEMICAL, AND PATHOLOGICAL-STUDIES OF 2 PATIENTS
GOLDMAN, JE
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RAPIN, I
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SUZUKI, K
[J]. ACTA NEUROPATHOLOGICA, 1980, 52 (03) : 189 - 202
[10] GORDON BA, 1975, CLIN RES, V22, pA740

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