Data di Pubblicazione:
2016
Citazione:
Current and novel aspects on the non-lysosomal β-Glucosylceramidase GBA2 / M. Aureli, M. Samarani, N. Loberto, G. Mancini, V. Murdica, E. Chiricozzi, A. Prinetti, R. Bassi, S. Sonnino. - In: NEUROCHEMICAL RESEARCH. - ISSN 0364-3190. - 41:1-2(2016 Feb), pp. 210-220.
Abstract:
Abstract The non-lysosomal b-glucosylceramidase
GBA2 (EC3.2.1.45, GH116) is ubiquitously expressed in
various mammal tissues and cell types where it catalyzes
the hydrolysis of glucosylceramide into glucose and ceramide.
Although it has been known for many years that the
central nervous system is the main site of GBA2 expression
and activity, little information has been available so far on
the role of this protein in the neuronal development,
senescence and homeostasis. In the present review, we
summarize the state-of-the art of this enzyme and in particular,
we focus on the current knowledge on its structure,
its physico-chemical properties and its subcellular localization.
Data on the involvement of GBA2 in physiopathological
processes are also described, with particular
emphasis on the studies that have indicated the direct
involvement of GBA2 in neuronal development and neurological
disorders. A discussion of some open questions
and future perspectives related to GBA2 are finally reported.
We conclude that further investigations on this
b-glucosylceramidase will provide new clues about the
physiology of the central nervous system.
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
b-Glucosylceramidase GBA2; glycosphingolipid; metabolism; hydrolase; plasma
Elenco autori:
M. Aureli, M. Samarani, N. Loberto, G. Mancini, V. Murdica, E. Chiricozzi, A. Prinetti, R. Bassi, S. Sonnino
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