Uncoupling N-acetylaspartate from brain pathology: implications for Canavan disease gene therapy
Uncoupling N-acetylaspartate from brain pathology: implications for Canavan disease gene therapy
About this item
Full title
Author / Creator
von Jonquieres, Georg , Spencer, Ziggy H. T. , Rowlands, Benjamin D. , Klugmann, Claudia B. , Bongers, Andre , Harasta, Anne E. , Parley, Kristina E. , Cederholm, Jennie , Teahan, Orla , Pickford, Russell , Delerue, Fabien , Ittner, Lars M. , Fröhlich, Dominik , McLean, Catriona A. , Don, Anthony S. , Schneider, Miriam , Housley, Gary D. , Rae, Caroline D. and Klugmann, Matthias
Publisher
Berlin/Heidelberg: Springer Berlin Heidelberg
Journal title
Language
English
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Publication information
Publisher
Berlin/Heidelberg: Springer Berlin Heidelberg
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More information
Scope and Contents
Contents
N
-Acetylaspartate (NAA) is the second most abundant organic metabolite in the brain, but its physiological significance remains enigmatic. Toxic NAA accumulation appears to be the key factor for neurological decline in Canavan disease—a fatal neurometabolic disorder caused by deficiency in the NAA-degrading enzyme aspartoacylase. To date clinic...
Alternative Titles
Full title
Uncoupling N-acetylaspartate from brain pathology: implications for Canavan disease gene therapy
Authors, Artists and Contributors
Author / Creator
Spencer, Ziggy H. T.
Rowlands, Benjamin D.
Klugmann, Claudia B.
Bongers, Andre
Harasta, Anne E.
Parley, Kristina E.
Cederholm, Jennie
Teahan, Orla
Pickford, Russell
Delerue, Fabien
Ittner, Lars M.
Fröhlich, Dominik
McLean, Catriona A.
Don, Anthony S.
Schneider, Miriam
Housley, Gary D.
Rae, Caroline D.
Klugmann, Matthias
Identifiers
Primary Identifiers
Record Identifier
TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_5756261
Permalink
https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_5756261
Other Identifiers
ISSN
0001-6322
E-ISSN
1432-0533
DOI
10.1007/s00401-017-1784-9
