Zebrafish: an emerging real-time model system to study Alzheimer's disease and neurospecific drug discovery
Zebrafish (Danio rerio) is emerging as an increasingly successful model for translational
research on human neurological disorders. In this review, we appraise the high degree of …
research on human neurological disorders. In this review, we appraise the high degree of …
The role of survival motor neuron protein (SMN) in protein homeostasis
H Chaytow, YT Huang, TH Gillingwater… - Cellular and Molecular …, 2018 - Springer
Ever since loss of survival motor neuron (SMN) protein was identified as the direct cause of
the childhood inherited neurodegenerative disorder spinal muscular atrophy, significant …
the childhood inherited neurodegenerative disorder spinal muscular atrophy, significant …
Neurocalcin delta suppression protects against spinal muscular atrophy in humans and across species by restoring impaired endocytosis
M Riessland, A Kaczmarek, S Schneider… - The American Journal of …, 2017 - cell.com
Homozygous SMN1 loss causes spinal muscular atrophy (SMA), the most common lethal
genetic childhood motor neuron disease. SMN1 encodes SMN, a ubiquitous housekeeping …
genetic childhood motor neuron disease. SMN1 encodes SMN, a ubiquitous housekeeping …
[HTML][HTML] Zebrafish models of human motor neuron diseases: advantages and limitations
PJ Babin, C Goizet, D Raldúa - Progress in neurobiology, 2014 - Elsevier
Motor neuron diseases (MNDs) are an etiologically heterogeneous group of disorders of
neurodegenerative origin, which result in degeneration of lower (LMNs) and/or upper motor …
neurodegenerative origin, which result in degeneration of lower (LMNs) and/or upper motor …
Disease mechanisms and therapeutic approaches in spinal muscular atrophy
S Tisdale, L Pellizzoni - Journal of Neuroscience, 2015 - Soc Neuroscience
Motor neuron diseases are neurological disorders characterized primarily by the
degeneration of spinal motor neurons, skeletal muscle atrophy, and debilitating and often …
degeneration of spinal motor neurons, skeletal muscle atrophy, and debilitating and often …
Single-cell mRNA profiling reveals cell-type-specific expression of neurexin isoforms
Neurexins are considered central organizers of synapse architecture that are implicated in
neuropsychiatric disorders. Expression of neurexins in hundreds of alternatively spliced …
neuropsychiatric disorders. Expression of neurexins in hundreds of alternatively spliced …
The power of human protective modifiers: PLS3 and CORO1C unravel impaired endocytosis in spinal muscular atrophy and rescue SMA phenotype
S Hosseinibarkooie, M Peters, L Torres-Benito… - The American Journal of …, 2016 - cell.com
Homozygous loss of SMN1 causes spinal muscular atrophy (SMA), the most common and
devastating childhood genetic motor-neuron disease. The copy gene SMN2 produces …
devastating childhood genetic motor-neuron disease. The copy gene SMN2 produces …
SMN control of RNP assembly: from post-transcriptional gene regulation to motor neuron disease
DK Li, S Tisdale, F Lotti, L Pellizzoni - Seminars in cell & developmental …, 2014 - Elsevier
At the post-transcriptional level, expression of protein-coding genes is controlled by a series
of RNA regulatory events including nuclear processing of primary transcripts, transport of …
of RNA regulatory events including nuclear processing of primary transcripts, transport of …
RNA-sequencing of a mouse-model of spinal muscular atrophy reveals tissue-wide changes in splicing of U12-dependent introns
TK Doktor, Y Hua, HS Andersen, S Brøner… - Nucleic acids …, 2017 - academic.oup.com
Abstract Spinal Muscular Atrophy (SMA) is a neuromuscular disorder caused by insufficient
levels of the Survival of Motor Neuron (SMN) protein. SMN is expressed ubiquitously and …
levels of the Survival of Motor Neuron (SMN) protein. SMN is expressed ubiquitously and …
Mitochondrial dysfunction in spinal muscular atrophy
Spinal muscular atrophy (SMA) is a devastating neuromuscular disorder caused by
recessive mutations in the SMN1 gene, globally affecting~ 8–14 newborns per 100,000. The …
recessive mutations in the SMN1 gene, globally affecting~ 8–14 newborns per 100,000. The …