Literature DB >> 17084653

Therapy for Gaucher disease: don't stop thinking about tomorrow.

Ellen Sidransky1, Mary E LaMarca, Edward I Ginns.   

Abstract

While enzyme replacement therapy for Gaucher disease has been widely used and appears to be an efficacious and safe treatment, this success should not be a reason for complacency. Other treatment strategies currently under consideration for patients with Gaucher disease include gene therapy, substrate reduction therapy and chaperone therapy. Furthermore, improvements in enzyme therapy could also have a significant clinical impact. Individuals with Gaucher disease and other lysosomal disorders will greatly benefit from continual refinement and optimization of the current therapy, as well as from the development of new treatment modalities that offer improvements in efficacy, cost, safety and availability.

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Year:  2006        PMID: 17084653     DOI: 10.1016/j.ymgme.2006.09.007

Source DB:  PubMed          Journal:  Mol Genet Metab        ISSN: 1096-7192            Impact factor:   4.797


  7 in total

1.  Inhibition of endoplasmic reticulum-associated degradation rescues native folding in loss of function protein misfolding diseases.

Authors:  Fan Wang; Wensi Song; Giovanna Brancati; Laura Segatori
Journal:  J Biol Chem       Date:  2011-10-17       Impact factor: 5.157

2.  Three classes of glucocerebrosidase inhibitors identified by quantitative high-throughput screening are chaperone leads for Gaucher disease.

Authors:  Wei Zheng; Janak Padia; Daniel J Urban; Ajit Jadhav; Ozlem Goker-Alpan; Anton Simeonov; Ehud Goldin; Douglas Auld; Mary E LaMarca; James Inglese; Christopher P Austin; Ellen Sidransky
Journal:  Proc Natl Acad Sci U S A       Date:  2007-08-01       Impact factor: 11.205

3.  Gaucher Disease-Induced Pluripotent Stem Cells Display Decreased Erythroid Potential and Aberrant Myelopoiesis.

Authors:  Judi A Sgambato; Tea Soon Park; Diana Miller; Leelamma M Panicker; Ellen Sidransky; Yu Lun; Ola Awad; Søren M Bentzen; Elias T Zambidis; Ricardo A Feldman
Journal:  Stem Cells Transl Med       Date:  2015-06-10       Impact factor: 6.940

4.  A high throughput glucocerebrosidase assay using the natural substrate glucosylceramide.

Authors:  Omid Motabar; Ehud Goldin; William Leister; Ke Liu; Noel Southall; Wenwei Huang; Juan J Marugan; Ellen Sidransky; Wei Zheng
Journal:  Anal Bioanal Chem       Date:  2011-10-28       Impact factor: 4.142

5.  Type 1 Gaucher disease: significant disease manifestations in "asymptomatic" homozygotes.

Authors:  Manisha Balwani; Laura Fuerstman; Ruth Kornreich; Lisa Edelmann; Robert J Desnick
Journal:  Arch Intern Med       Date:  2010-09-13

Review 6.  Hematopoietic stem cell expansion and gene therapy.

Authors:  Korashon Lynn Watts; Jennifer Adair; Hans-Peter Kiem
Journal:  Cytotherapy       Date:  2011-11       Impact factor: 5.414

7.  Optimization and validation of two miniaturized glucocerebrosidase enzyme assays for high throughput screening.

Authors:  Daniel J Urban; Wei Zheng; Ozlem Goker-Alpan; Ajit Jadhav; Mary E Lamarca; James Inglese; Ellen Sidransky; Christopher P Austin
Journal:  Comb Chem High Throughput Screen       Date:  2008-12       Impact factor: 1.339

  7 in total

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