http://www.cnr.it/ontology/cnr/individuo/prodotto/ID4174
The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiation (Articolo in rivista)
- Type
- Label
- The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiation (Articolo in rivista) (literal)
- Anno
- 2005-01-01T00:00:00+01:00 (literal)
- Alternative label
Gabanella F., Carissimi C., Usiello A. and Pellizzoni L. (2005)
The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiation
in Human molecular genetics (Print)
(literal)
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- Gabanella F., Carissimi C., Usiello A. and Pellizzoni L. (literal)
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- Pubblicazione su rivista internazionale (literal)
- Note
- ISI Web of Science (WOS) (literal)
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- Titolo
- The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiation (literal)
- Abstract
- Spinal muscular atrophy (SMA) is a lethal neuromuscular disease caused by reduced levels of expression
of the survival motor neuron (SMN) protein. SMN is part of a macromolecular complex essential for the
assembly of the small nuclear ribonucleoproteins (snRNPs) that carry out pre-mRNA splicing. Although
the SMN complex has the potential to control the pathway of snRNP biogenesis, it is not known whether
SMN function in snRNP assembly is regulated. Here, we analyze SMN interactions and function in mouse
tissues and show that, when normalized per cell number, similar levels of the SMN complex are expressed
throughout the ontogenesis of the central nervous system (CNS). Strikingly, however, SMN function in
snRNP assembly in extracts does not correlate with its expression levels and it varies greatly both among
tissues and during development. The highest levels of SMN activity are found during the embryonic and
early postnatal development of the CNS and are followed by a sharp decrease to a basal level, which is
then maintained throughout life. This downregulation takes place in the spinal cord earlier than in the
brain and coincides with the onset of myelination. Using model cell systems and pulse-labeling experiments,
we further show that SMN activity and snRNP synthesis are strongly downregulated upon neuronal as well as
myogenic differentiation, and linked to the rate of global transcription of postmitotic neurons and myotubes.
These results demonstrate that the SMN complex activity in snRNP assembly is regulated and point to a
differential requirement for SMN function during development and cellular differentiation. (literal)
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