UPTAKE AND METABOLISM OF RADIOACTIVELY LABELED SPHINGOMYELIN IN CULTURED SKIN FIBROBLASTS FROM CONTROLS AND PATIENTS WITH NIEMANN-PICK DISEASE AND OTHER LYSOSOMAL STORAGE DISEASES

被引:63
作者
KUDOH, T [1 ]
VELKOFF, MA [1 ]
WENGER, DA [1 ]
机构
[1] UNIV COLORADO,SCH MED,DEPT PEDIAT,DENVER,CO 80262
关键词
D O I
10.1016/0005-2760(83)90084-X
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:82 / 92
页数:11
相关论文
共 31 条
[1]  
BARTLETT GR, 1959, J BIOL CHEM, V234, P466
[2]   METABOLISM OF SPHINGOMYELIN BY INTACT CULTURED FIBROBLASTS - DIFFERENTIATION OF NIEMANN-PICK DISEASE TYPE-A AND TYPE-B [J].
BEAUDET, AL ;
MANSCHRECK, AA .
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1982, 105 (01) :14-19
[3]   BIOSYNTHESIS OF SPHINGOMYELIN FROM ERYTHRO-CERAMIDES AND PHOSPHATIDYLCHOLINE BY A MICROSOMAL CHOLINEPHOSPHOTRANSFERASE [J].
BERNERT, JT ;
ULLMAN, MD .
BIOCHIMICA ET BIOPHYSICA ACTA, 1981, 666 (01) :99-109
[4]   SPHINGOMYELINASE DEFECT IN NIEMANN-PICK DISEASE, TYPE-C, FIBROBLASTS [J].
BESLEY, GTN .
FEBS LETTERS, 1977, 80 (01) :71-74
[5]  
Brady R. O., 1983, METABOLIC BASIS INHE, P831
[6]  
CALLAHAN JW, 1981, LYSOSOMES LYSOSOMAL, P205
[7]   NIEMANN-PICK DISEASE, TYPE-C - EVIDENCE FOR THE DEFICIENCY OF AN ACTIVATING FACTOR STIMULATING SPHINGOMYELIN AND GLUCOCEREBROSIDE DEGRADATION [J].
CHRISTOMANOU, H .
HOPPE-SEYLERS ZEITSCHRIFT FUR PHYSIOLOGISCHE CHEMIE, 1980, 361 (10) :1489-1502
[8]  
GATT S, 1980, J BIOL CHEM, V255, P3371
[9]   PROGRESSIVE PARESIS OF VERTICAL GAZE IN LIPID STORAGE DISEASE [J].
GROVER, WD ;
NAIMAN, JL .
NEUROLOGY, 1971, 21 (09) :896-+
[10]   DIAGNOSIS OF METACHROMATIC LEUKODYSTROPHY, KRABBE DISEASE, AND FARBER DISEASE AFTER UPTAKE OF FATTY-ACID LABELED CEREBROSIDE SULFATE INTO CULTURED SKIN FIBROBLASTS [J].
KUDOH, T ;
WENGER, DA .
JOURNAL OF CLINICAL INVESTIGATION, 1982, 70 (01) :89-97