Smn1 Antibody

1. Survival Motor Neuron (SMN) protein is required for normal mouse liver development PMID:27698380
2. Widespread intron retention, particularly of minor U12 introns, in the spinal cord of mice 30 d after SMA induction. PMID:28270613
3. A proteomic profile of embryonic stem cells with low smn protein revealed thematic changes consistent with the developmental dysfunction seen in the pathophysiological development of patients with spinal muscular atrophy. Pathways associated with mRNA spicing, protein translation, post-translational modification and perhaps most striking, mitochondrial function and specifically mitochondrial dysfunction were highlighted. PMID:27145767
4. Loganin is capable of increas-ing the SMN protein level under SMN-deficient conditions both inin vitro and in vivo models of spinal muscular atrophy via Akt/mTOR pathway. PMID:27241020
5. miR-431 expression was highly increased, and a number of its putative mRNA targets were significantly downregulated in motor neurons after SMN loss. Further, we found that miR-431 regulates motor neuron neurite length by targeting several molecules previously identified to play a role in motor neuron axon outgrowth, including chondrolectin PMID:27005422
6. To determine the dependence of oligodendrocyte (OL)on the Smn protein(SMN1), we utilized the Smn-/-;SMN2 (severe) mouse model. Our data suggest that despite the multi-functionality and ubiquitous expression of the Smn protein, it does not play a key role in myelination of the CNS, at least in the context of spinal muscular atrophy pathogenesis. PMID:28069797
7. our studies show that this G-motif represents a novel and essential determinant for axonal localization of the Anxa2 mRNA mediated by the SMN complex. PMID:28258160
8. A long non-coding RNA (lncRNA) that arises from the antisense strand of SMN, SMN-AS1, is enriched in neurons and transcriptionally represses SMN expression by recruiting the epigenetic Polycomb repressive complex-2. PMID:28017471
9. SMN1 expression restoration is curative in a spinal muscular atrophy model mice. PMID:27907033
10. Survival motor neuron 1, and survival motor neuron 2, depletion results in increased alternative splicing events. PMID:27736905
11. these results demonstrate that SMN deficiency impacts spleen development and suggests a potential role for immunological development in Spinal muscular atrophy. PMID:28062667
12. Itch monoubiquitinates SMN and monoubiquitination of SMN plays an important role in regulating its cellular localization. PMID:26908624
13. muscle does not appear to require high levels of SMN above what is produced by two copies of SMN2 PMID:26276812
14. Findings demonstrate that high expression of SMN in the motor neuron is both necessary and sufficient for proper function of the motor unit. In addition, SMN high expression in neurons and glia has a major impact on survival. PMID:26206889
15. This study identifies pathways related to the function of Smn and associated with differential motor unit vulnerability, thus presenting a number of exciting targets for future therapeutic development. PMID:26374403
16. Smn complex deficiency caused constipation, delayed gastric emptying, slow intestinal transit and reduced colonic motility. PMID:25859009
17. Primary cell culture and two different SMA model mice to demonstrate that reduced levels of Smn lead to a profound disruption in the expression of myogenic genes. PMID:24691550
18. Results suggest that SMN plays a role in the maintenance of pluripotent embryonic stem cells and neuronal differentiation in mice. PMID:24633826
19. AAV9-mediated SMN gene therapy elicits cure for spinal muscular atrophy. PMID:25358252
20. Data show that changes in U12 introns-dependent splicing become apparent after prolonged/extensive survival motor neuron proteins SMN depletion. PMID:25692239
21. This work both reveals a new autoregulatory pathway governing SMN expression, and identifies a new mechanism through which SMN can modulate specific mRNA expression via Gemin5. PMID:25911097
22. SMN is involved in the axonal translocation of hnRNP R and hnRNP R-bound RNA/protein complexes. PMID:25338097
23. Findings are consistent with a role for SMN in myotube formation through effects on muscle differentiation and cell motility. PMID:24760765
24. Data indicate that Smn deficiency affects the subcellular transcriptome in both the somatodendritic and axonal regions of motoneurons impairing transcripts with selected functions. PMID:25246652
25. Findings suggest that carriers of SMN1 mutations and/or deletions may be at an increased risk of developing pancreatic and glucose metabolism defects. PMID:24497575
26. Data support a role for SMN in the regulation of mRNA localization and axonal transport through its interaction with mRNA-binding proteins such as IMP1 PMID:23897586
27. It improves neuromuscular function and motor neuron survival in mutant SOD1 mice. PMID:24210254
28. SMN1 is essential for U7 biogenesis and histone mRNA processing. PMID:24332368
29. sregulation of UBA1 and subsequent ubiquitination pathways led to beta-catenin accumulation in a model of mutant SMN spinal muscular atrophy. PMID:24590288
30. Restoration of SMN to Emx-1 expressing cortical neurons is not sufficient to provide benefit to a severe mouse model of Spinal Muscular Atrophy. PMID:23512182
31. enhanced Survival Motoneuron protein has a role in neuromuscular junction maturation PMID:24463453
32. a new mechanism for regulating SMN levels and provides new insight into the roles of U1A in 3' processing of mRNAs. PMID:24362020
33. Study shows that neuronal aggregates formed by mutant FUS protein may aberrantly sequester survival motor neuron protein and concomitantly cause a reduction of SMN levels in the axon, leading to axonal defects. PMID:23681068
34. Smn protein reduction causes an increment of the autophagosome number as well as of the autophagy-related proteins Beclin1 and LC3-II. PMID:23788043
35. Low SMN levels might result in localization deficiencies of mRNAs required for axonogenesis. PMID:24152552
36. SAHA significantly increases SMN levels and also increased vascular density in SMA mice (P<0.05), suggesting that the vascular defect in SMA mice is amenable to SAHA treatment. PMID:23583590
37. Overexpressing the SMN protein in mutant SOD1 mice, a model of familial ALS, alleviates this phenomenon, most likely in a cell-autonomous manner, and significantly mitigates the loss of motor neurons in the spinal cord and in culture dishes. PMID:22581780
38. Expressing SMN pan-neuronally in a mouse model of severe spinal muscular atrophy results in a four-fold increase in survival. PMID:23029491
39. Data suggest a critical role of aurvival of motor neuron SMN1 and SMN2 proteins in the intrinsic regulation of muscle differentiation and suggest that abnormal muscle development contributes to the manifestation of spinal muscular atrophy (SMA) symptoms. PMID:22705478
40. SMN plays distinct roles in muscle, neuromuscular junctions, and motor neuron somal synapses PMID:22723710
41. This study identified a critical threshold of Smn that dictates onset of SMA in the intermediate Smn(2B/-) mouse model. PMID:22071333
42. Hyper-phosphorylation of profilin2a is the molecular link between SMN and the ROCK pathway repressing neurite outgrowth in neuronal cells. PMID:21920940
43. SMN is essential for the normal postnatal maturation of motor nerve terminals. PMID:22022549
44. comparison of systemic versus CNS restoration of SMN in a severe mouse model PMID:21979052
45. This study demonistrated that the spinal muscular atrophy mouse model, SMADelta7, displays altered axonal transport without global neurofilament alterations PMID:21681521
46. Prolactin increases SMN expression and survival in a mouse model of severe spinal muscular atrophy via the STAT5 pathway PMID:21785216
47. even in severe SMA, timely reinstatement of the SMN protein may halt the progression of the disease PMID:21785219
48. Data show that SMN and HuD form a complex in spinal motor axons, and that both interact with cpg15 mRNA in neurons. PMID:21652774
49. Involvement of SMN in formation of stress granules may play an important role in cell survival. PMID:21234798
50. Mouse survival motor neuron alleles that mimic SMN2 splicing and are inducible rescue embryonic lethality early in development but not late [SMN2] PMID:21249120

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UNIGENE: Mm.2025

KEGG: mmu:20595

STRING: 10090.ENSMUSP00000022147