BETA-GALACTOSIDASE DEFICIENT-TYPE MUCOLIPODOSIS - COMPLEMENTATION STUDY OF NEURAMINIDASE IN SOMATIC-CELL HYBRIDS

被引:15
作者
KATO, T
OKADA, S
YUTAKA, T
INUI, K
YABUUCHI, H
CHIYO, H
FURUYAMA, J
OKADA, Y
机构
[1] OSAKA UNIV HOSP, DEPT PEDIAT, FUKUSHIMA KU, OSAKA, FUKUSHIMA 553, JAPAN
[2] OSAKA UNIV, MICROBIAL DIS RES INST, SUITA, OSAKA 565, JAPAN
[3] HYOGO MED COLL, DEPT GENET, NISHINOMIYA, HYOGO 660, JAPAN
关键词
D O I
10.1016/0006-291X(79)90590-4
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
By cell fusion with polyethylene glycol (PEG) a remarkable increase of neuraminidase activity was observed in the fused cells between ML-II and other two neuraminidase deficient disorders, ML-Gal (mucolipidosis, galactosidase deficient type) and ML-I (mucolipidosis I). No complementation was found in the combination of ML-I and ML-Gal. This result suggests that ML-I and ML-Gal may be allelic mutations and belong to the same genetic disorder as a primary neuraminidase deficiency. © 1979.
引用
收藏
页码:114 / 117
页数:4
相关论文
共 14 条
  • [1] MUCOLIPIDOSIS-1 - INCREASED SIALIC-ACID CONTENT AND DEFICIENCY OF ANALPHA-N-ACETYLNEURAMINIDASE IN CULTURED FIBROBLASTS
    CANTZ, M
    GEHLER, J
    SPRANGER, J
    [J]. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1977, 74 (02) : 732 - 738
  • [2] IMPROVED TECHNIQUES FOR INDUCTION OF MAMMALIAN-CELL HYBRIDIZATION BY POLYETHYLENE-GLYCOL
    DAVIDSON, RL
    GERALD, PS
    [J]. SOMATIC CELL GENETICS, 1976, 2 (02): : 165 - 176
  • [3] DURAND P, 1977, HELV PAEDIATR ACTA, V32, P391
  • [4] RAPID AND SENSITIVE ASSAY FOR NEURAMINIDASE - APPLICATION TO CULTURED FIBROBLASTS
    FRISCH, A
    NEUFELD, EF
    [J]. ANALYTICAL BIOCHEMISTRY, 1979, 95 (01) : 222 - 227
  • [5] LOWDEN JA, 1979, AM J HUM GENET, V31, P1
  • [6] LOWRY OH, 1951, J BIOL CHEM, V193, P265
  • [7] COMPLEMENTATION OF BETA-GALACTOSIDASE IN FUSED CELLS OF MUCOLIPIDOSIS-II WITH ANOTHER VARIANTS OF BETA-GALACTOSIDASE DEFICIENCY USING NEW SINGLE CELL ENZYME ASSAY
    OKADA, S
    KATO, T
    YABUUCHI, H
    OKADA, Y
    [J]. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1979, 88 (02) : 559 - 562
  • [8] CASE OF NEURAMINIDASE DEFICIENCY ASSOCIATED WITH A PARTIAL BETA-GALACTOSIDASE DEFECT - CLINICAL, BIOCHEMICAL AND RADIOLOGICAL STUDIES
    OKADA, S
    YUTAKA, T
    KATO, T
    IKEHARA, C
    YABUUCHI, H
    OKAWA, M
    INUI, M
    CHIYO, H
    [J]. EUROPEAN JOURNAL OF PEDIATRICS, 1979, 130 (04) : 239 - 249
  • [9] FLUOROMETRIC ASSAY OF NEURAMINIDASE WITH A SODIUM (4-METHYLUMBELLIFERYL-ALPHA-D-N-ACETYLNEURAMINATE) SUBSTRATE
    POTIER, M
    MAMELI, L
    BELISLE, M
    DALLAIRE, L
    MELANCON, SB
    [J]. ANALYTICAL BIOCHEMISTRY, 1979, 94 (02) : 287 - 296
  • [10] MUCOLIPIDOSIS-I - SIALIDOSIS
    SPRANGER, J
    GEHLER, J
    CANTZ, M
    [J]. AMERICAN JOURNAL OF MEDICAL GENETICS, 1977, 1 (01): : 21 - 29