Literature DB >> 6816485

Galactosialidosis (beta-galactosidase-neuraminidase deficiency): a possible role of serine-thiol proteases in the degradation of beta-galactosidase molecules.

H Sakuraba, T Aoyagi, Y Suzuki.   

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Year:  1982        PMID: 6816485     DOI: 10.1016/0009-8981(82)90257-1

Source DB:  PubMed          Journal:  Clin Chim Acta        ISSN: 0009-8981            Impact factor:   3.786


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  12 in total

1.  Adrenoleukodystrophy and beta-galactosidase deficiency: patient and carrier.

Authors:  I Goto; T Yoshimura; T Kobayashi; Y Kuroiwa
Journal:  J Neurol       Date:  1986-10       Impact factor: 4.849

2.  Characterization and purification of human beta-galactosidase overexpressed in recombinant baculovirus-infected Spodoptera frugiperda cells.

Authors:  K Itoh; A Oshima; H Sakuraba; Y Suzuki
Journal:  J Inherit Metab Dis       Date:  1991       Impact factor: 4.982

3.  GM1-Gangliosidosis: a molecular abnormality of acid beta-galactosidase in fibroblasts.

Authors:  T Furuya; Y Suzuki
Journal:  J Inherit Metab Dis       Date:  1984       Impact factor: 4.982

4.  Stable expression of protective protein/cathepsin A-green fluorescent protein fusion genes in a fibroblastic cell line from a galactosialidosis patient. Model system for revealing the intracellular transport of normal and mutated lysosomal enzymes.

Authors:  Y Naganawa; K Itoh; M Shimmoto; S Kamei; K Takiguchi; H Doi; H Sakuraba
Journal:  Biochem J       Date:  1999-06-01       Impact factor: 3.857

5.  Beta-galactosidase-deficient human fibroblasts: uptake and processing of the exogenous precursor enzyme expressed by stable transformant COS cells.

Authors:  A Oshima; K Itoh; Y Nagao; H Sakuraba; Y Suzuki
Journal:  Hum Genet       Date:  1990-10       Impact factor: 4.132

6.  Beta-galactosidase-deficient mouse as an animal model for GM1-gangliosidosis.

Authors:  J Matsuda; O Suzuki; A Oshima; A Ogura; Y Noguchi; Y Yamamoto; T Asano; K Takimoto; K Sukegawa; Y Suzuki; M Naiki
Journal:  Glycoconj J       Date:  1997-09       Impact factor: 2.916

7.  Human beta-galactosidase gene mutations in GM1-gangliosidosis: a common mutation among Japanese adult/chronic cases.

Authors:  K Yoshida; A Oshima; M Shimmoto; Y Fukuhara; H Sakuraba; N Yanagisawa; Y Suzuki
Journal:  Am J Hum Genet       Date:  1991-08       Impact factor: 11.025

8.  Chemical chaperone therapy for brain pathology in G(M1)-gangliosidosis.

Authors:  Junichiro Matsuda; Osamu Suzuki; Akihiro Oshima; Yoshie Yamamoto; Akira Noguchi; Kazuhiro Takimoto; Masayuki Itoh; Yuji Matsuzaki; Yosuke Yasuda; Seiichiro Ogawa; Yuko Sakata; Eiji Nanba; Katsumi Higaki; Yoshimi Ogawa; Lika Tominaga; Kousaku Ohno; Hiroyuki Iwasaki; Hiroshi Watanabe; Roscoe O Brady; Yoshiyuki Suzuki
Journal:  Proc Natl Acad Sci U S A       Date:  2003-12-15       Impact factor: 11.205

9.  Intracellular processing and maturation of mutant gene products in hereditary beta-galactosidase deficiency (beta-galactosidosis).

Authors:  A Oshima; K Yoshida; K Itoh; R Kase; H Sakuraba; Y Suzuki
Journal:  Hum Genet       Date:  1994-02       Impact factor: 4.132

10.  Protective protein gene mutations in galactosialidosis.

Authors:  M Shimmoto; Y Fukuhara; K Itoh; A Oshima; H Sakuraba; Y Suzuki
Journal:  J Clin Invest       Date:  1993-06       Impact factor: 14.808

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