Raquel L Lieberman

Summary

Affiliation: Georgia Institute of Technology
Country: USA

Publications

  1. pmc Biophysical characterization of the olfactomedin domain of myocilin, an extracellular matrix protein implicated in inherited forms of glaucoma
    Susan D Orwig
    School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, Georgia, United States of America
    PLoS ONE 6:e16347. 2011
  2. pmc A Guided Tour of the Structural Biology of Gaucher Disease: Acid-β-Glucosidase and Saposin C
    Raquel L Lieberman
    School of Chemistry and Biochemistry, Institute for Bioscience and Bioengineering, Georgia Institute of Technology, 901 Atlantic Drive NW Atlanta, GA 30332 0400, USA
    Enzyme Res 2011:973231. 2011
  3. doi request reprint Crystallization chaperone strategies for membrane proteins
    Raquel L Lieberman
    School of Chemistry and Biochemistry, Institute for Bioscience and Bioengineering, Georgia Institute of Technology, 901 Atlantic Drive NW, Atlanta, GA 30332, USA
    Methods 55:293-302. 2011
  4. pmc Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability
    Raquel L Lieberman
    Structural Neurology Lab at the Center for Neurologic Diseases, Brigham and Women s Hospital and Harvard Medical School, 77 Avenue Louis Pasteur, Boston, Massachusetts 02115, USA
    Biochemistry 48:4816-27. 2009
  5. pmc The glaucoma-associated olfactomedin domain of myocilin forms polymorphic fibrils that are constrained by partial unfolding and peptide sequence
    Shannon E Hill
    School of Chemistry and Biochemistry, Georgia Institute of Technology, 901 Atlantic Drive Northwest, Atlanta, GA 30332 0400, USA
    J Mol Biol 426:921-35. 2014
  6. pmc The glaucoma-associated olfactomedin domain of myocilin is a novel calcium binding protein
    Rebecca K Donegan
    School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, Georgia 30332 0400, USA
    J Biol Chem 287:43370-7. 2012
  7. pmc Amyloid fibril formation by the glaucoma-associated olfactomedin domain of myocilin
    Susan D Orwig
    School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, GA 30332, USA
    J Mol Biol 421:242-55. 2012
  8. pmc Rescue of glaucoma-causing mutant myocilin thermal stability by chemical chaperones
    J Nicole Burns
    Georgia Institute of Technology, Atlanta, 30332, USA
    ACS Chem Biol 5:477-87. 2010
  9. pmc From rhomboid function to structure and back again
    Raquel L Lieberman
    Center for Neurologic Diseases, Brigham and Women s Hospital, Harvard Medical School, 77 Avenue Louis Pasteur, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 104:8199-200. 2007
  10. pmc The stability of myocilin olfactomedin domain variants provides new insight into glaucoma as a protein misfolding disorder
    J Nicole Burns
    School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, Georgia 30332 0400, United States
    Biochemistry 50:5824-33. 2011

Collaborators

Detail Information

Publications15

  1. pmc Biophysical characterization of the olfactomedin domain of myocilin, an extracellular matrix protein implicated in inherited forms of glaucoma
    Susan D Orwig
    School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, Georgia, United States of America
    PLoS ONE 6:e16347. 2011
    ..The data presented here inform new testable hypotheses for interactions with specific TEM components, and will assist in design of therapeutic agents for myocilin glaucoma...
  2. pmc A Guided Tour of the Structural Biology of Gaucher Disease: Acid-β-Glucosidase and Saposin C
    Raquel L Lieberman
    School of Chemistry and Biochemistry, Institute for Bioscience and Bioengineering, Georgia Institute of Technology, 901 Atlantic Drive NW Atlanta, GA 30332 0400, USA
    Enzyme Res 2011:973231. 2011
    ..This review focuses on the details of these structures to highlight salient common and disparate features that contribute to our current state of knowledge of this complex orphan disease...
  3. doi request reprint Crystallization chaperone strategies for membrane proteins
    Raquel L Lieberman
    School of Chemistry and Biochemistry, Institute for Bioscience and Bioengineering, Georgia Institute of Technology, 901 Atlantic Drive NW, Atlanta, GA 30332, USA
    Methods 55:293-302. 2011
    ....
  4. pmc Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability
    Raquel L Lieberman
    Structural Neurology Lab at the Center for Neurologic Diseases, Brigham and Women s Hospital and Harvard Medical School, 77 Avenue Louis Pasteur, Boston, Massachusetts 02115, USA
    Biochemistry 48:4816-27. 2009
    ..Taken together, these results provide insight into how GCase and alpha-Gal A are thermodynamically stabilized by iminosugars and suggest strategies for the development of new pharmacological chaperones for lysosomal storage disorders...
  5. pmc The glaucoma-associated olfactomedin domain of myocilin forms polymorphic fibrils that are constrained by partial unfolding and peptide sequence
    Shannon E Hill
    School of Chemistry and Biochemistry, Georgia Institute of Technology, 901 Atlantic Drive Northwest, Atlanta, GA 30332 0400, USA
    J Mol Biol 426:921-35. 2014
    ..Our study places myoc-OLF within the larger landscape of the amylome and provides insight into the diversity of myoc-OLF aggregation that plays a role in glaucoma pathogenesis...
  6. pmc The glaucoma-associated olfactomedin domain of myocilin is a novel calcium binding protein
    Rebecca K Donegan
    School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, Georgia 30332 0400, USA
    J Biol Chem 287:43370-7. 2012
    ....
  7. pmc Amyloid fibril formation by the glaucoma-associated olfactomedin domain of myocilin
    Susan D Orwig
    School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, GA 30332, USA
    J Mol Biol 421:242-55. 2012
    ....
  8. pmc Rescue of glaucoma-causing mutant myocilin thermal stability by chemical chaperones
    J Nicole Burns
    Georgia Institute of Technology, Atlanta, 30332, USA
    ACS Chem Biol 5:477-87. 2010
    ....
  9. pmc From rhomboid function to structure and back again
    Raquel L Lieberman
    Center for Neurologic Diseases, Brigham and Women s Hospital, Harvard Medical School, 77 Avenue Louis Pasteur, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 104:8199-200. 2007
  10. pmc The stability of myocilin olfactomedin domain variants provides new insight into glaucoma as a protein misfolding disorder
    J Nicole Burns
    School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, Georgia 30332 0400, United States
    Biochemistry 50:5824-33. 2011
    ..Our work provides the first quantitative demonstration of compromised stability among many identified OLF variants and places myocilin glaucoma in the context of other diseases of protein misfolding...
  11. ncbi request reprint Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease
    Raquel L Lieberman
    Structural Neurology Lab, Brigham and Women s Hospital and Harvard Medical School, 77 Ave Louis Pasteur, Boston, Massachusetts 02115, USA
    Nat Chem Biol 3:101-7. 2007
    ..The design of small molecules that stabilize substrate-bound conformations of mutant proteins may be a general therapeutic strategy for diseases caused by protein misfolding and mistrafficking...
  12. pmc Binding of 3,4,5,6-tetrahydroxyazepanes to the acid-β-glucosidase active site: implications for pharmacological chaperone design for Gaucher disease
    Susan D Orwig
    School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, Georgia 30332, United States
    Biochemistry 50:10647-57. 2011
    ..Our results emphasize the importance of the conformational variability of the GCase active site in the design of competitive inhibitors as PCs for Gaucher disease...
  13. doi request reprint Determination of soluble and membrane protein structures by X-ray crystallography
    Raquel L Lieberman
    School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, GA, USA
    Methods Mol Biol 955:475-93. 2013
    ....
  14. pmc Ligands for glaucoma-associated myocilin discovered by a generic binding assay
    Susan D Orwig
    School of Chemistry and Biochemistry, Georgia Institute of Technology, 901 Atlantic Drive NW, Atlanta, Georgia 30332 0400, United States
    ACS Chem Biol 9:517-25. 2014
    ..Our compounds set the stage for a new chemical probe approach to clarify the biological function of wild-type myocilin and represent lead therapeutic compounds for diminishing intracellular sequestration of toxic mutant myocilin. ..
  15. pmc Membrane-embedded protease poses for photoshoot
    Raquel L Lieberman
    Center for Neurologic Diseases, Brigham and Women s Hospital, Harvard Medical School, 77 Avenue Louis Pasteur, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 104:401-2. 2007