Neurochondrin interacts with the SMN protein suggesting a novel mechanism for Spinal Muscular Atrophy pathology
| Autor: | Thompson, Luke W., Morrison, Kim D., Shirran, Sally L., Groen, Ewout J. N., Gillingwater, Tom H., Botting, Catherine H., Sleeman, Judith E. |
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| Přispěvatelé: | Medical Research Council, The Wellcome Trust, University of St Andrews. School of Biology, University of St Andrews. School of Chemistry, University of St Andrews. EaSTCHEM, University of St Andrews. Biomedical Sciences Research Complex, University of St Andrews. Institute of Behavioural and Neural Sciences |
| Rok vydání: | 2017 |
| Předmět: |
QH301 Biology
animal diseases snRNPs SmN 0302 clinical medicine Cells Cultured R2C 0303 health sciences Neurochondrin SMN Complex Proteins Translation (biology) SMA SMN 3. Good health Cell biology BDC RC0321 Neuroscience. Biological psychiatry. Neuropsychiatry Research Article Neurite snRNP Nerve Tissue Proteins Context (language use) QH426 Genetics Biology Sm protein Muscular Atrophy Spinal QH301 03 medical and health sciences Splicing factor medicine Humans QH426 030304 developmental biology Messenger RNA DAS Spinal muscular atrophy medicine.disease Molecular biology nervous system diseases nervous system Spinal Muscular Atrophy RC0321 Sm proteins SmB SMN1 030217 neurology & neurosurgery SMN2 |
| Zdroj: | Journal of Cell Science |
| DOI: | 10.1101/183640 |
| Popis: | Spinal muscular atrophy (SMA) is an inherited neurodegenerative condition caused by a reduction in the amount of functional survival motor neuron (SMN) protein. SMN has been implicated in transport of mRNA in neural cells for local translation. We previously identified microtubule-dependent mobile vesicles rich in SMN and SNRPB, a member of the Sm family of small nuclear ribonucleoprotein (snRNP)-associated proteins, in neural cells. By comparing the interactomes of SNRPB and SNRPN, a neural-specific Sm protein, we now show that the essential neural protein neurochondrin (NCDN) interacts with Sm proteins and SMN in the context of mobile vesicles in neurites. NCDN has roles in protein localisation in neural cells and in maintenance of cell polarity. NCDN is required for the correct localisation of SMN, suggesting they may both be required for formation and transport of trafficking vesicles. NCDN may have potential as a therapeutic target for SMA together with, or in place of the targeting of SMN expression. This article has an associated First Person interview with the first author of the paper. Highlighted Article: The essential neural protein neurochondrin interacts with the spinal muscular atrophy (SMA) protein SMN in cell lines and in mice. This might be relevant to the molecular pathology of SMA. |
| Databáze: | OpenAIRE |
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