JUVENILE NEURONAL CEROID-LIPOFUSCINOSIS - CHARACTERIZATION OF THE DYSLIPOPROTEINEMIA AND DEMONSTRATION OF MEMBRANE PHOSPHOLIPID AND PHOSPHOLIPID-DEPENDENT SIGNAL-TRANSDUCTION ABNORMALITIES IN CULTURED SKIN FIBROBLASTS

被引：10
作者：
BENNETT, MJ
POIRIER, SF
CHERN, L
GAYTON, AR
HOSKING, GP
LE, NA
MAJUMDAR, S
KORCHAK, HM
机构：
[1] RYEGATE CHILDRENS CTR,SHEFFIELD,ENGLAND
[2] EMORY UNIV,DEPT MED,ATLANTA,GA 30322
来源：
JOURNAL OF INHERITED METABOLIC DISEASE | 1993年 / 16卷 / 02期
关键词：
D O I：
10.1007/BF00710272
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
[No abstract available]
引用
收藏
页码：308 / 311
页数：4
相关论文
共 9 条
[1]   DECREASED ERYTHROCYTE AND PLATELET PHOSPHOLIPIDS AND FATTY-ACIDS IN JUVENILE NEURONAL CEROID-LIPOFUSCINOSIS (BATTEN DISEASE) [J].
BENNETT, MJ ;
GALLOWAY, JH ;
CARTWRIGHT, IJ ;
GILLIS, WS ;
HOSKING, GP .
NEUROPEDIATRICS, 1990, 21 (04) :202-205
[2]   ABNORMAL LYSOSOMAL CATHEPSIN ACTIVITIES IN LEUKOCYTES AND CULTURED SKIN FIBROBLASTS IN LATE INFANTILE, BUT NOT IN JUVENILE NEURONAL CEROID-LIPOFUSCINOSIS (BATTEN DISEASE) [J].
BENNETT, MJ ;
CHERN, L ;
CARPENTER, KH ;
SLADKY, JT .
CLINICA CHIMICA ACTA, 1992, 208 (1-2) :111-117
[3]  
CORKEY BE, 1991, J CLIN INVEST, V87, P782
[4]  
CRYER DR, 1986, J LIPID RES, V28, P195
[5]   BATTEN DISEASE (SPIELMEYER-VOGT DISEASE, JUVENILE ONSET NEURONAL CEROID-LIPOFUSCINOSIS) GENE (CLN3) MAPS TO HUMAN CHROMOSOME-16 [J].
GARDINER, M ;
SANDFORD, A ;
DEADMAN, M ;
POULTON, J ;
COOKSON, W ;
REEDERS, S ;
JOKIAHO, I ;
PELTONEN, L ;
EIBERG, H ;
JULIER, C .
GENOMICS, 1990, 8 (02) :387-390
[6]   LIPID ABNORMALITIES IN BATTENS DISEASE [J].
GILLIS, WS ;
BENNETT, MJ ;
GALLOWAY, JH ;
CARTWRIGHT, IJ ;
HOSKING, GP ;
SMITH, CML .
JOURNAL OF INHERITED METABOLIC DISEASE, 1987, 10 :329-332
[7]  
HALL NA, 1991, DEV NEUROSCI, V13, P399
[8]   MITOCHONDRIAL ATP SYNTHASE SUBUNIT C STORAGE IN THE CEROID-LIPOFUSCINOSES (BATTEN DISEASE) [J].
PALMER, DN ;
FEARNLEY, IM ;
WALKER, JE ;
HALL, NA ;
LAKE, BD ;
WOLFE, LS ;
HALTIA, M ;
MARTINUS, RD ;
JOLLY, RD .
AMERICAN JOURNAL OF MEDICAL GENETICS, 1992, 42 (04) :561-567
[9]  
PALMER DN, 1990, LIPOFUSCIN CEROID PI, P211
1