Literature DB >> 5338605

Demonstration of a deficiency of glucocerebroside-cleaving enzyme in Gaucher's disease.

R O Brady, J N Kanfer, R M Bradley, D Shapiro.   

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Year:  1966        PMID： 5338605      PMCID： PMC292783          DOI： 10.1172/JCI105417

Source DB:  PubMed          Journal:  J Clin Invest        ISSN： 0021-9738            Impact factor:   14.808


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  8 in total

1.  METABOLISM OF GLUCOCEREBROSIDES. II. EVIDENCE OF AN ENZYMATIC DEFICIENCY IN GAUCHER'S DISEASE.

Authors:  R O BRADY; J N KANFER; D SHAPIRO
Journal:  Biochem Biophys Res Commun       Date:  1965-01-18       Impact factor: 3.575

2.  Neurological signs in a juvenile form of Gaucher's disease.

Authors:  K M HERRLIN; P O HILLBORG
Journal:  Acta Paediatr       Date:  1962-03       Impact factor: 2.299

3.  Enzymic oxidation of cerebrosides: studies on Gaucher's disease.

Authors:  B W AGRANOFF; N RADIN; W SUOMI
Journal:  Biochim Biophys Acta       Date:  1962-02-12

4.  Cerebroside synthesis in Gaucher's disease.

Authors:  E G TRAMS; R O BRADY
Journal:  J Clin Invest       Date:  1960-10       Impact factor: 14.808

5.  A reinvestigation of the cerebroside deposited in Gaucher's disease.

Authors:  A ROSENBERG; E CHARGAFF
Journal:  J Biol Chem       Date:  1958-12       Impact factor: 5.157

6.  A simple method for the isolation and purification of total lipides from animal tissues.

Authors:  J FOLCH; M LEES; G H SLOANE STANLEY
Journal:  J Biol Chem       Date:  1957-05       Impact factor: 5.157

7.  Turnover of the fatty acids of rat brain gangliosides, glycerophosphatides, cerebrosides, and sulfatides as a function of age.

Authors:  Y Kishimoto; W E Davies; N S Radin
Journal:  J Lipid Res       Date:  1965-10       Impact factor: 5.922

8.  THE METABOLISM OF GLUCOCEREBROSIDES. I. PURIFICATION AND PROPERTIES OF A GLUCOCEREBROSIDE-CLEAVING ENZYME FROM SPLEEN TISSUE.

Authors:  R O BRADY; J KANFER; D SHAPIRO
Journal:  J Biol Chem       Date:  1965-01       Impact factor: 5.157
  8 in total
  89 in total

1.  Histone deacetylase inhibitors prevent the degradation and restore the activity of glucocerebrosidase in Gaucher disease.

Authors:  Jie Lu; Chunzhang Yang; Masako Chen; Donald Y Ye; Russell R Lonser; Roscoe O Brady; Zhengping Zhuang
Journal:  Proc Natl Acad Sci U S A       Date:  2011-12-12       Impact factor: 11.205

2.  Benefits from unearthing "a biochemical Rosetta Stone".

Authors:  Roscoe O Brady
Journal:  J Biol Chem       Date:  2010-10-28       Impact factor: 5.157

3.  Ultrasensitive in situ visualization of active glucocerebrosidase molecules.

Authors:  Martin D Witte; Wouter W Kallemeijn; Jan Aten; Kah-Yee Li; Anneke Strijland; Wilma E Donker-Koopman; Adrianus M C H van den Nieuwendijk; Boris Bleijlevens; Gertjan Kramer; Bogdan I Florea; Berend Hooibrink; Carla E M Hollak; Roelof Ottenhoff; Rolf G Boot; Gijsbert A van der Marel; Herman S Overkleeft; Johannes M F G Aerts
Journal:  Nat Chem Biol       Date:  2010-10-31       Impact factor: 15.040

4.  Bone marrow involvement in Gaucher disease at MRI : what long-term evolution can we expect under enzyme replacement therapy?

Authors:  Benjamin Fedida; Sébastien Touraine; Jerôme Stirnemann; Nadia Belmatoug; Jean-Denis Laredo; David Petrover
Journal:  Eur Radiol       Date:  2015-04-16       Impact factor: 5.315

5.  Improved management of lysosomal glucosylceramide levels in a mouse model of type 1 Gaucher disease using enzyme and substrate reduction therapy.

Authors:  John Marshall; Kerry Anne McEachern; Wei-Lien Chuang; Elizabeth Hutto; Craig S Siegel; James A Shayman; Greg A Grabowski; Ronald K Scheule; Diane P Copeland; Seng H Cheng
Journal:  J Inherit Metab Dis       Date:  2010-03-25       Impact factor: 4.982

6.  The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms.

Authors:  Richard A Steet; Stephen Chung; Brandon Wustman; Allan Powe; Hung Do; Stuart A Kornfeld
Journal:  Proc Natl Acad Sci U S A       Date:  2006-08-31       Impact factor: 11.205

7.  Decreased glucocerebrosidase activity in Gaucher disease parallels quantitative enzyme loss due to abnormal interaction with TCP1 and c-Cbl.

Authors:  Jie Lu; Jeffrey Chiang; Rajiv R Iyer; Eli Thompson; Christine R Kaneski; David S Xu; Chunzhang Yang; Masako Chen; Richard J Hodes; Russell R Lonser; Roscoe O Brady; Zhengping Zhuang
Journal:  Proc Natl Acad Sci U S A       Date:  2010-11-22       Impact factor: 11.205

Review 8.  Treatment of lysosomal storage disorders : progress with enzyme replacement therapy.

Authors:  Marianne Rohrbach; Joe T R Clarke
Journal:  Drugs       Date:  2007       Impact factor: 9.546

9.  Distinguishing the differences in β-glycosylceramidase folds, dynamics, and actions informs therapeutic uses.

Authors:  Fredj Ben Bdira; Marta Artola; Herman S Overkleeft; Marcellus Ubbink; Johannes M F G Aerts
Journal:  J Lipid Res       Date:  2018-10-02       Impact factor: 5.922

10.  Gaucher disease: the effects of phosphatidylserine on glucocerebrosidase from normal and Gaucher fibroblasts.

Authors:  F Y Choy
Journal:  Hum Genet       Date:  1984       Impact factor: 4.132
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