CYSTEAMINE DEPLETES CYSTINOTIC LEUKOCYTE GRANULAR FRACTIONS OF CYSTINE BY THE MECHANISM OF DISULFIDE INTERCHANGE

被引：69

作者：

GAHL, WA

TIETZE, F

BUTLER, JD

SCHULMAN, JD

机构：

[1] NIADDKD, SECT INTERMEDIARY METAB, BETHESDA, MD 20205 USA

[2] NICHHD, BIOCHEM GENET SECT, BETHESDA, MD 20205 USA

来源：

BIOCHEMICAL JOURNAL | 1985年 / 228卷 / 03期

关键词：

D O I：

10.1042/bj2280545

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：545 / 550

页数：6

共 17 条

[1] PANTETHINE AND CYSTAMINE DEPLETE CYSTINE FROM CYSTINOTIC FIBROBLASTS VIA EFFLUX OF CYSTEAMINE-CYSTEINE MIXED DISULFIDE [J].

BUTLER, JD ;

ZATZ, M .

JOURNAL OF CLINICAL INVESTIGATION, 1984, 74 (02) :411-416

[2] CHARACTERISTICS OF CYSTINE COUNTER-TRANSPORT IN NORMAL AND CYSTINOTIC LYSOSOME-RICH LEUKOCYTE GRANULAR FRACTIONS [J].

GAHL, WA ;

TIETZE, F ;

BASHAN, N ;

BERNARDINI, I ;

RAIFORD, D ;

SCHULMAN, JD .

BIOCHEMICAL JOURNAL, 1983, 216 (02) :393-400

[3]

GAHL WA, 1982, J BIOL CHEM, V257, P9570

[4] CYSTINE TRANSPORT IS DEFECTIVE IN ISOLATED LEUKOCYTE LYSOSOMES FROM PATIENTS WITH CYSTINOSIS [J].

GAHL, WA ;

BASHAN, N ;

TIETZE, F ;

BERNARDINI, I ;

SCHULMAN, JD .

SCIENCE, 1982, 217 (4566) :1263-1265

[5]

GOLDMAN H, 1970, LANCET, V1, P811

[6]

GOLDMAN H, 1974, CLIN RES, V22, pA740

[7] DECREASE IN FREE CYSTINE CONTENT OF CULTURED CYSTINOTIC FIBROBLASTS BY ASCORBIC-ACID [J].

KROLL, WA ;

SCHNEIDER, JA .

SCIENCE, 1974, 186 (4168) :1040-1042

[8]

LOWRY OH, 1951, J BIOL CHEM, V193, P265

[9] PANTETHEINASE ACTIVITY AND CYSTEAMINE CONTENT IN CYSTINOTIC AND NORMAL FIBROBLASTS AND LEUKOCYTES [J].

ORLOFF, S ;

BUTLER, JD ;

TOWNE, D ;

MUKHERJEE, AB ;

SCHULMAN, JD .

PEDIATRIC RESEARCH, 1981, 15 (07) :1063-1067

[10]

PISONI RL, 1985, FED PROC, V44, P1805

← 1 2 →