HEXOSAMINIDASE A DEFICIENT ADULTS - PRESENCE OF ALPHA-CHAIN PRECURSOR IN CULTURED SKIN FIBROBLASTS

被引:15
作者
FRISCH, A
BARAM, D
NAVON, R
机构
[1] TEL AVIV UNIV,SACKLER FAC MED,DEPT HUMAN GENET,TEL HASHOMER,ISRAEL
[2] CHAIM SHEBA MED CTR,IL-52621 TEL HASHOMER,ISRAEL
关键词
D O I
10.1016/0006-291X(84)91624-3
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
收藏
页码:101 / 107
页数:7
相关论文
共 9 条
[1]  
ARGOV Z, 1983, UNPUB
[2]  
HASILIK A, 1980, J BIOL CHEM, V255, P4937
[3]  
NAVON R, 1976, AM J HUM GENET, V28, P339
[4]  
NAVON R, 1973, AM J HUM GENET, V25, P287
[5]  
OBRIEN JS, 1983, METABOLIC BASIS INHE, P945
[6]  
PADEH B, 1971, ISRAEL J MED SCI, V7, P259
[7]   SYNTHESIS OF BETA-HEXOSAMINIDASE IN CELL-FREE TRANSLATION AND IN INTACT FIBROBLASTS - AN INSOLUBLE PRECURSOR ALPHA-CHAIN IN A RARE FORM OF TAY-SACHS DISEASE [J].
PROIA, RL ;
NEUFELD, EF .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1982, 79 (20) :6360-6364
[8]   ADULT (CHRONIC) GM2 GANGLIOSIDOSIS - ATYPICAL SPINOCEREBELLAR DEGENERATION IN A JEWISH SIBSHIP [J].
RAPIN, I ;
SUZUKI, K ;
SUZUKI, K ;
VALSAMIS, MP .
ARCHIVES OF NEUROLOGY, 1976, 33 (02) :120-130
[9]   CHRONIC GM2 GANGLIOSIDOSIS MASQUERADING AS ATYPICAL FRIEDREICH ATAXIA - CLINICAL, MORPHOLOGIC, AND BIOCHEMICAL-STUDIES OF 9 CASES [J].
WILLNER, JP ;
GRABOWSKI, GA ;
GORDON, RE ;
BENDER, AN ;
DESNICK, RJ .
NEUROLOGY, 1981, 31 (07) :787-798