ENZYME THERAPY IN GM2 GANGLIOSIDOSIS - CARBOHYDRATE-SPECIFIC HUMAN BETA-HEXOSAMINIDASE UPTAKE BY FELINE LIVER

被引：1
|
作者：
RATTAZZI, MC
机构：
[1] CHILDRENS HOSP,BUFFALO,NY 14222
[2] SUNY BUFFALO,DEPT PEDIAT,BUFFALO,NY 14214
来源：
PEDIATRIC RESEARCH | 1978年 / 12卷 / 04期
关键词：
D O I：
10.1203/00006450-197804001-00561
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
引用
收藏
页码：456 / 456
页数：1
相关论文
共 50 条
  • [21] Chaperone Therapy for GM2 Gangliosidosis: Effects of Pyrimethamine on β-Hexosaminidase Activity in Sandhoff Fibroblasts
    Chiricozzi, Elena
    Niemir, Natalia
    Aureli, Massimo
    Magini, Alessandro
    Loberto, Nicoletta
    Prinetti, Alessandro
    Bassi, Rosaria
    Polchi, Alice
    Emiliani, Carla
    Caillaud, Catherine
    Sonnino, Sandro
    MOLECULAR NEUROBIOLOGY, 2014, 50 (01) : 159 - 167
  • [22] Chaperone Therapy for GM2 Gangliosidosis: Effects of Pyrimethamine on β-Hexosaminidase Activity in Sandhoff Fibroblasts
    Elena Chiricozzi
    Natalia Niemir
    Massimo Aureli
    Alessandro Magini
    Nicoletta Loberto
    Alessandro Prinetti
    Rosaria Bassi
    Alice Polchi
    Carla Emiliani
    Catherine Caillaud
    Sandro Sonnino
    Molecular Neurobiology, 2014, 50 : 159 - 167
  • [23] THE HUMAN GM2 ACTIVATOR PROTEIN - A SUBSTRATE SPECIFIC COFACTOR OF BETA-HEXOSAMINIDASE-A
    MEIER, EM
    SCHWARZMANN, G
    FURST, W
    SANDHOFF, K
    JOURNAL OF BIOLOGICAL CHEMISTRY, 1991, 266 (03) : 1879 - 1887
  • [24] Therapeutic Potential of Intracerebroventricular Replacement of Modified Human β-Hexosaminidase B for GM2 Gangliosidosis
    Matsuoka, Kazuhiko
    Tamura, Tomomi
    Tsuji, Daisuke
    Dohzono, Yukie
    Kitakaze, Keisuke
    Ohno, Kazuki
    Saito, Seiji
    Sakuraba, Hitoshi
    Itoh, Kohji
    MOLECULAR THERAPY, 2011, 19 (06) : 1017 - 1024
  • [25] ENZYME REPLACEMENT IN FELINE GM2 GANGLIOSIDOSIS - REDUCTION OF GLYCOLIPID STORAGE IN VISCERAL ORGANS
    RATTAZZI, MC
    APPEL, AM
    BAKER, HJ
    AMERICAN JOURNAL OF HUMAN GENETICS, 1980, 32 (06) : A51 - A51
  • [26] In Cellulo Examination of a Beta-Alpha Hybrid Construct of Beta-Hexosaminidase A Subunits, Reported to Interact with the GM2 Activator Protein and Hydrolyze GM2 Ganglioside
    Sinici, Incilay
    Yonekawa, Sayuri
    Tkachyova, Ilona
    Gray, Steven J.
    Samulski, R. Jude
    Wakarchuk, Warren
    Mark, Brian L.
    Mahuran, Don J.
    PLOS ONE, 2013, 8 (03):
  • [27] CHARACTERIZATION OF HEX-S, MAJOR RESIDUAL BETA-HEXOSAMINIDASE ACTIVITY IN TYPE-O GM2 GANGLIOSIDOSIS (SANDHOFF-JATZKEWITZ DISEASE)
    IKONNE, JU
    RATTAZZI, MC
    DESNICK, RJ
    AMERICAN JOURNAL OF HUMAN GENETICS, 1975, 27 (05) : 639 - 650
  • [28] CHRONIC GM2-GANGLIOSIDOSIS (CGM2) - CHARACTERIZATION OF THE RESIDUAL BETA-HEXOSAMINIDASE (HEX)-A ACTIVITY
    GRABOWSKI, GA
    CONZELMAN, E
    SANDHOFF, K
    DESNICK, RJ
    AMERICAN JOURNAL OF HUMAN GENETICS, 1981, 33 (06) : A44 - A44
  • [29] JUVENILE GM2 GANGLIOSIDOSIS CAUSED BY SUBSTITUTION OF HISTIDINE FOR ARGININE AT POSITION-499 OR POSITION-504 OF THE ALPHA-SUBUNIT OF BETA-HEXOSAMINIDASE
    PAW, BH
    MOSKOWITZ, SM
    UHRHAMMER, N
    WRIGHT, N
    KABACK, MM
    NEUFELD, EF
    JOURNAL OF BIOLOGICAL CHEMISTRY, 1990, 265 (16) : 9452 - 9457
  • [30] Protease-resistant modified human β-hexosaminidase B ameliorates symptoms in GM2 gangliosidosis model
    Kitakaze, Keisuke
    Mizutani, Yasumichi
    Sugiyama, Eiji
    Tasaki, Chikako
    Tsuji, Daisuke
    Maita, Nobuo
    Hirokawa, Takatsugu
    Asanuma, Daisuke
    Kamiya, Mako
    Sato, Kohei
    Setou, Mitsutoshi
    Urano, Yasuteru
    Togawa, Tadayasu
    Otaka, Akira
    Sakuraba, Hitoshi
    Itoh, Kohji
    JOURNAL OF CLINICAL INVESTIGATION, 2016, 126 (05): : 1691 - 1703
12345