N-ACETYLNEURAMINIC ACID ACCUMULATION IN A BUOYANT LYSOSOMAL FRACTION OF CULTURED FIBROBLASTS FROM PATIENTS WITH INFANTILE GENERALIZED N-ACETYLNEURAMINIC ACID STORAGE DISEASE

被引：16
|
作者：
HILDRETH, J
SACKS, L
HANCOCK, LW
机构：
[1] UNIV CHICAGO,JOSEPH P KENNEDY JR MENTAL RETARDAT RES CTR,DEPT MOLEC BIOL,CHICAGO,IL 60637
[2] UNIV CHICAGO,JOSEPH P KENNEDY JR MENTAL RETARDAT RES CTR,DEPT BIOCHEM,CHICAGO,IL 60637
来源：
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS | 1986年 / 139卷 / 02期
关键词：
D O I：
10.1016/S0006-291X(86)80066-3
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：838 / 844
页数：7
相关论文
共 50 条
  • [1] N-ACETYLNEURAMINIC ACID AND SIALOGLYCOCONJUGATE METABOLISM IN FIBROBLASTS FROM A PATIENT WITH GENERALIZED N-ACETYLNEURAMINIC ACID STORAGE DISEASE
    HANCOCK, LW
    HORWITZ, AL
    DAWSON, G
    BIOCHIMICA ET BIOPHYSICA ACTA, 1983, 760 (01) : 42 - 52
  • [2] N-ACETYLNEURAMINIC ACID STORAGE DISEASE
    BAUMKOTTER, J
    CANTZ, M
    MENDLA, K
    BAUMANN, W
    FRIEBOLIN, H
    GEHLER, J
    SPRANGER, J
    HUMAN GENETICS, 1985, 71 (02) : 155 - 159
  • [3] IMPAIRED PROTEOLYTIC PROCESSING OF LYSOSOMAL N-ACETYL-BETA-HEXOSAMINIDASE IN CULTURED FIBROBLASTS FROM PATIENTS WITH INFANTILE GENERALIZED N-ACETYLNEURAMINIC ACID STORAGE DISEASE
    HANCOCK, LW
    RICKETTS, JP
    HILDRETH, J
    BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1988, 152 (01) : 83 - 92
  • [4] Characterization of a novel N-acetylneuraminic acid lyase favoring N-acetylneuraminic acid synthesis
    Ji, Wenyan
    Sun, Wujin
    Feng, Jinmei
    Song, Tianshun
    Zhang, Dalu
    Ouyang, Pingkai
    Gu, Zhen
    Xie, Jingjing
    SCIENTIFIC REPORTS, 2015, 5
  • [5] An efficient process for production of N-acetylneuraminic acid using N-acetylneuraminic acid aldolase
    Mahmoudian, M
    Noble, D
    Drake, CS
    Middleton, RF
    Montgomery, DS
    Piercey, JE
    Ramlakhan, D
    Todd, M
    Dawson, MJ
    ENZYME AND MICROBIAL TECHNOLOGY, 1997, 20 (05) : 393 - 400
  • [6] CONFIGURATION OF N-ACETYLNEURAMINIC ACID
    BRUG, J
    PAERELS, GB
    NATURE, 1958, 182 (4643) : 1159 - 1160
  • [7] SYNTHESIS OF N-ACETYLNEURAMINIC ACID
    CORNFORTH, JW
    FIRTH, ME
    GOTTSCHALK, A
    BIOCHEMICAL JOURNAL, 1958, 68 : 57 - &
  • [8] GENERALIZED N-ACETYLNEURAMINIC STORAGE WITH SIALURIA
    PHILIPPART, M
    KAMENSKY, E
    CANCILLA, P
    CALLAHAN, J
    ZEILSTRA, K
    NAKATANI, S
    FARRIAUX, JP
    PEDIATRIC RESEARCH, 1974, 8 (04) : 393 - 393
  • [9] SYNTHESIS OF N-ACETYLNEURAMINIC ACID
    RINDERKNECHT, H
    REBANE, T
    EXPERIENTIA, 1963, 19 (07): : 342 - &
  • [10] DEFECTIVE LYSOSOMAL EGRESS OF FREE SIALIC-ACID (N-ACETYLNEURAMINIC ACID) IN FIBROBLASTS OF PATIENTS WITH INFANTILE FREE SIALIC-ACID STORAGE DISEASE
    TIETZE, F
    SEPPALA, R
    RENLUND, M
    HOPWOOD, JJ
    HARPER, GS
    THOMAS, GH
    GAHL, WA
    JOURNAL OF BIOLOGICAL CHEMISTRY, 1989, 264 (26) : 15316 - 15322
12345