{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Locatelli D"],"funding":["Telethon"],"pagination":["25782-94"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC3406665"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["287(31)"],"pubmed_abstract":["Spinal muscular atrophy is a fatal genetic disease of motoneurons due to loss of full-length survival of motor neuron protein, the main product of the disease gene SMN1. Axonal SMN (a-SMN) is an alternatively spliced isoform of SMN1, generated by retention of intron 3. To study a-SMN function, we generated cellular clones for the expression of the protein in mouse motoneuron-like NSC34 cells. The model was instrumental in providing evidence that a-SMN decreases cell growth and plays an important role in the processes of axon growth and cellular motility. In our conditions, low levels of a-SMN expression were sufficient to trigger the observed biological effects, which were not modified by further increasing the amounts of the expressed protein. Differential transcriptome analysis led to th"],"journal":["The Journal of biological chemistry"],"pubmed_title":["Human axonal survival of motor neuron (a-SMN) protein stimulates axon growth, cell motility, C-C motif ligand 2 (CCL2), and insulin-like growth factor-1 (IGF1) production."],"pmcid":["PMC3406665"],"funding_grant_id":["GGP07223"],"pubmed_authors":["Fratelli M","Barzago MM","Capra S","Terao M","D'Errico P","Kurosaki M","Lupi M","Zanetti A","Uggetti A","Battaglia GS","Locatelli D","Garattini E"],"additional_accession":[]},"is_claimable":false,"name":"Human axonal survival of motor neuron (a-SMN) protein stimulates axon growth, cell motility, C-C motif ligand 2 (CCL2), and insulin-like growth factor-1 (IGF1) production.","description":"Spinal muscular atrophy is a fatal genetic disease of motoneurons due to loss of full-length survival of motor neuron protein, the main product of the disease gene SMN1. Axonal SMN (a-SMN) is an alternatively spliced isoform of SMN1, generated by retention of intron 3. To study a-SMN function, we generated cellular clones for the expression of the protein in mouse motoneuron-like NSC34 cells. The model was instrumental in providing evidence that a-SMN decreases cell growth and plays an important role in the processes of axon growth and cellular motility. In our conditions, low levels of a-SMN expression were sufficient to trigger the observed biological effects, which were not modified by further increasing the amounts of the expressed protein. Differential transcriptome analysis led to th","dates":{"release":"2012-01-01T00:00:00Z","publication":"2012 Jul","modification":"2025-04-04T23:29:25.877Z","creation":"2019-06-05T17:28:51Z"},"accession":"S-EPMC3406665","cross_references":{"pubmed":["22669976"],"doi":["10.1074/jbc.M112.362830"]}}