Rehabilitating mutant GCase

Jennifer N Rauch; Jason E Gestwicki

doi:10.1016/j.chembiol.2014.07.010

Rehabilitating mutant GCase

Chem Biol. 2014 Aug 14;21(8):919-20. doi: 10.1016/j.chembiol.2014.07.010.

Authors

Jennifer N Rauch¹, Jason E Gestwicki²

Affiliations

¹ Department of Pharmaceutical Chemistry, Institute for Neurodegenerative Disease, University of California at San Francisco, 675 Nelson Rising Lane, San Francisco, CA 94158, USA.
² Department of Pharmaceutical Chemistry, Institute for Neurodegenerative Disease, University of California at San Francisco, 675 Nelson Rising Lane, San Francisco, CA 94158, USA. Electronic address: [email protected].

PMID: 25126987
DOI: 10.1016/j.chembiol.2014.07.010

Abstract

Gaucher's disease is a hereditary deficiency of the enzyme β-glucocerebrosidase (GCase) that is most commonly treated by enzyme replacement therapy. In this issue of Chemistry & Biology, Tan and colleagues search for alternative ways to rehabilitate mutant GCase by understanding how it interacts with the proteostasis network.

Publication types

Comment

MeSH terms

Endoplasmic Reticulum / metabolism*
Gaucher Disease / enzymology*
Glucosylceramidase / genetics*
Glucosylceramidase / metabolism*
HSP40 Heat-Shock Proteins / metabolism*
Humans
Mutant Proteins / metabolism*

Substances

DNAJB11 protein, human
HSP40 Heat-Shock Proteins
Mutant Proteins
Glucosylceramidase