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1 SMA is caused by low levels of the survival motor neuron
2 SMA is characterized by alpha-lower motor neuron loss an
3 SMA is characterized by loss of motor neurons, but the u
4 SMA is subdivided into four main types, with type I bein
5 SMA patients have deletions and other mutations of the s
6 SMA results from reduced levels of the ubiquitously expr
7 SMA therapeutics development efforts have focused on ide
8 SMA-affected individuals harbor low SMN expression from
9 SMA-causing missense mutations that block multimerizatio
16 We report a novel small molecule acceptor (SMA) named FTTB-PDI4 obtained via ring-fusion between th
17 ceptor (A-D-A)-type small molecule acceptor (SMA), and an indaceno[1,2-b:5,6-b']dithiophene-based SMA
18 ibes the preparation of styrene maleic acid (SMA) co-polymer to extract membrane proteins from prokar
20 istochemical studies of smooth muscle actin (SMA), D2-40, CD34, and glucose transporter 1 (GLUT-1).
21 ility, apoptosis, alpha-smooth muscle actin (SMA), fibronectin (FN) production, and collagen remodeli
24 trons, in the spinal cord of mice 30 d after SMA induction, which was then rescued by a therapeutic A
25 nd -bundling protein, fully protects against SMA in SMN1-deleted individuals carrying 3-4 SMN2 copies
27 functional stability of shape memory alloys (SMAs), especially for high temperature applications.
28 healing via promotion of expression of alpha SMA and TGF beta, neovascularization and re-epithelializ
29 pression of alpha-smooth muscle actin (alpha-SMA) and Collagen I were reduced as was the activity of
34 ynthesis of alpha-smooth muscle actin (alpha-SMA), collagen 1 (COL1), and fibronectin (FN) in respons
35 neration of alpha-smooth muscle actin (alpha-SMA)-positive myofibroblasts that produced ECM proteins,
38 mity to the alpha-smooth muscle actin (alpha-SMA+) area within mild fibrosis regions; while in severe
39 erentiated (alpha-smooth muscle actin [alpha-SMA](+)) fibrocytes were increased in asthmatic patients
40 and significantly reduces collagen and alpha-SMA expression in an animal model of liver fibrosis.
42 Cells positive for MMP-9, MMP-13, and alpha-SMA expression were present at the areas of epithelial i
45 umbers of circulating alpha-SMA(+) and alpha-SMA(+)CXCR4(+) fibrocytes were increased in asthmatic pa
46 liver IL-6, IL-17A, IL-17F, TGF-beta1, alpha-SMA, TGR5, NTCP, OATP1a1, and ileum ASBT and decreased l
48 In addition, numbers of circulating alpha-SMA(+) and alpha-SMA(+)CXCR4(+) fibrocytes were increase
49 creased infiltration of EP4 expressing alpha-SMA myofibroblasts, identifying a potential mechanism of
52 In contrast, a dramatic increase in alpha-SMA(+), EP4(+) double-positive cells were observed in EP
55 to the effects of hemoglobin inducing alpha-SMA expressions in cultured pericytes and brain slices v
56 n increase in the myofibroblast marker alpha-SMA, production and assembly of FN, and contraction of c
57 downregulated, while the expression of alpha-SMA and vimentin was upregulated in diabetic cataract ti
60 at the TSS of transcript variant 2 of alpha-SMA, which correlated with an increase in alpha-SMA prot
61 re, by targeting the eNOS and pericyte alpha-SMA phenotype, our present data may shed new light on th
62 nhibition of FAK activation blocks the alpha-SMA and collagen expression, and inhibits the formation
65 ith SMN2 splicing modification to ameliorate SMA and demonstrates the promise of combinatorial ASOs f
66 tly, NCALD knockdown effectively ameliorates SMA-associated pathological defects across species, incl
67 tified plastin 3 (PLS3) overexpression as an SMA protective modifier in humans and showed that SMN de
71 ajority of cold-induced beige adipocytes and SMA-marked progenitors appeared essential for beiging.
72 stive, GLUT-1-negative endothelial cells and SMA-positive pericytes arranged in macro- or microlobule
78 CS intensity when conditioning PMv, rM1 and SMA at a 40-ms ISI, with larger effects after PMv condit
81 pain, whereas the supplementary motor area (SMA) and pre-SMA are specifically associated with higher
82 l cortex (PFC) and supplementary motor area (SMA) during emotion regulation, although only change in
83 etermined that the supplementary motor area (SMA) plays an important role in the interlimb transfer o
84 rtex (rPMd) or the supplementary motor area (SMA) prior to the TS at various CS-TS inter-stimulus int
86 aneurysm of the superior mesenteric artery (SMA) with a diameter of 2.2 cm was found incidentally on
87 neuromuscular deficits and diseases such as SMA, as well as for next generation prosthetics, utilizi
92 underlying cause of spinal muscular atrophy (SMA) is a deficiency of the survival motor neuron (SMN)
112 motoneuron disease spinal muscular atrophy (SMA) is caused by low levels of the survival motor neuro
114 NIFICANCE STATEMENT Spinal muscular atrophy (SMA) is caused by the loss of motor neurons, but astrocy
117 Infantile-onset spinal muscular atrophy (SMA) is the most common genetic cause of infant mortalit
121 in a mouse model of spinal muscular atrophy (SMA), a reduction in proprioceptive synaptic drive leads
123 motoneuron disease spinal muscular atrophy (SMA), motor axons fail to form the normal extent of axon
126 romuscular disorder spinal muscular atrophy (SMA), the most common inherited killer of infants, is ca
127 us SMN1 loss causes spinal muscular atrophy (SMA), the most common lethal genetic childhood motor neu
130 d an indaceno[1,2-b:5,6-b']dithiophene-based SMA, namely IDTN, by end-capping with the naphthyl fused
131 Defects in contact interactions between SMA motoneurons and astrocytes impair synaptogenesis see
132 erhemispheric interactions between bilateral SMA play an instrumental role in CE and that the structu
133 fiber density in tracts connecting bilateral SMA were negatively correlated with and predictive of tr
134 cles of the hind-paw were vulnerable in both SMA and ALS, with a loss of neuronal innervation and shr
136 located patients (2:1 with stratification by SMA type and centre) to receive olesoxime (10 mg/kg per
138 ron markers at day 0 among study groups (CM, SMA, and CC groups) and at day 28 between children in ea
140 MA) activity tracked task-set control costs, SMA activity tracked response-set control costs, and bas
141 this for a high temperature ternary Ti-Pd-Cr SMA by achieving both a small DeltaT and high transforma
142 Our results are not limited to Ti-Pd-Cr SMAs but potentially provide a strategy for searching fo
143 in the peripheral nervous system by creating SMA mice selectively overexpressing SMN in myelinating S
144 onstrates modifier impact in three different SMA animal models, and suggests a potential combinatoria
146 sed for future development of drugs for DM1, SMA, and other chronic diseases where GSK-3beta inhibiti
147 immunoglobulin light-chain variable domain, SMA, associated with AL amyloidosis, were investigated b
148 of SMA, NMR studies were performed for each SMA-like point mutant of LEN followed by in silico analy
150 mplications of this therapeutic landmark for SMA therapeutics and discuss how future developments wil
152 rst precise molecularly targeted therapy for SMA (nusinersen), and a pivotal proof of principle that
154 e is currently no FDA-approved treatment for SMA, early therapeutic efforts have focused on testing r
156 ologous light-chain, LEN, which differs from SMA at eight positions but is non-amyloidogenic in vivo,
158 rget of SMN and show that motor neurons from SMA mouse models have reduced levels ofGAP43mRNA and pro
159 ntron retention in all examined tissues from SMA mice, and that U12-dependent intron retention is ind
160 er motoneurons, astrocytes or both were from SMA mice compared with those in WT motoneurons coculture
162 fects are observed in the most severe Type I SMA patients and most of the widely used SMA mouse model
166 increase the level of SMN protein by >50% in SMA patient-derived fibroblasts at concentrations of <16
167 ved macrophages, were found to be altered in SMA spleens even in pre-symptomatic post-natal day 2 ani
168 Ns) are the most obviously affected cells in SMA, recent evidence suggest dysfunction in multiple cel
169 e specific defects in the motor circuitry in SMA is still unclear, but SMN has also been implicated i
172 ne whether the immune system is comprised in SMA, we analyzed the spleen and immunological components
175 we report that MCP1 levels are decreased in SMA mice and that replacement of deficient MCP1 increase
177 a critical role for mitochondrial defects in SMA pathogenesis and suggest a novel target for improvin
180 s a novel aspect of astrocyte dysfunction in SMA and indicates a possible approach for improving moto
182 ) SMNDelta7, provides a protective effect in SMA model mice and human motor neuron cell culture syste
184 , as NCALD knockdown improves endocytosis in SMA models and ameliorates pharmacologically induced end
185 analysis of survival to combined endpoint in SMA infants with 2 copies of SMN2 indicated a median age
190 es expressed in motor neurons is involved in SMA pathogenesis, but increasing evidence indicates that
199 s is a major cellular mechanism perturbed in SMA and emphasize the power of protective modifiers for
201 action potential (CMAP) decreased rapidly in SMA infants, whereas MFS in all healthy infants rapidly
202 development and point to a role for RBPs in SMA.SIGNIFICANCE STATEMENT In zebrafish models of the mo
204 Importantly, this activity is reduced in SMA patients, raising the possibility that the aetiology
206 We propose that defects in intron removal in SMA promote DNA damage in part through the formation of
207 c in vivo, we find that multiple residues in SMA clustered around the N-terminus and CDR loops experi
208 DNF expression does not play a major role in SMA pathology as viral-mediated GDNF re-expression did n
210 and astrocytes impair synaptogenesis seen in SMA pathology, possibly due to the disruption of the Eph
211 at miR-146a was significantly upregulated in SMA induced pluripotent stem cell (iPSC)-derived astrocy
214 ccessful isolation of membrane proteins into SMA lipid particles (SMALPs) allows the proteins to rema
215 Herein, we introduce a novel ca. 1.6 kDa SMA-based polymer with styrene:maleic acid moieties that
216 hite matter fibers connecting right and left SMA predicts the benefit that an individual derives from
219 llele C (C (+/+)) mouse recapitulates a mild SMA-like phenotype and offers an ideal system to monitor
221 ificant reduction in spleen size in multiple SMA mouse models and a pathological reduction in red pul
222 en SMN function and the distal neuromuscular SMA phenotype is an incorrectly spliced transcript or tr
227 oved understanding of the molecular basis of SMA, disease pathogenesis, natural history, and recognit
232 Our findings uncover novel hallmarks of SMA disease progression and link SMN to general male inf
233 opment of the neurodegenerative hallmarks of SMA, implying a differential role of SMN in myeloid cell
234 ping the dynamic conformational landscape of SMA, NMR studies were performed for each SMA-like point
235 leotide (ASO)-based inducible mouse model of SMA to investigate the SMN-specific transcriptome change
240 his isoform of Agrin in the motor neurons of SMA model mice increases muscle fiber size, enhances the
244 ent introns is mitigated by ASO treatment of SMA mice and that many transcriptional changes are rever
245 otential of these molecules for treatment of SMA, our work has wide-ranging implications in understan
252 SMA are commercially available, so far only SMA variants with a 2:1 and 3:1 styrene-to-maleic acid r
256 pendently of intensity, CS over pIFG and pre-SMA (but not over a control site) inhibited MEPs at an I
257 s the supplementary motor area (SMA) and pre-SMA are specifically associated with higher-level cognit
260 including pre-supplementary motor area (pre-SMA), inferior frontal gyrus, caudate, and subthalamic n
262 ) was administered over ipsilateral pIFG/pre-SMA sites before the TS at different CS-TS inter-stimulu
263 m and predictive models, mediated by the pre-SMA and its connectivity in frontostriatal circuits.
264 e STN on continue-evoked activity in the pre-SMA was predicted by interindividual differences in risk
265 In particular, the pIFG -but not the pre-SMA- exerts intensity-dependent modulatory influences ov
268 or Neurocalcin delta (NCALD) as a protective SMA modifier in five asymptomatic SMN1-deleted individua
269 y identifies a previously unknown protective SMA modifier in humans, demonstrates modifier impact in
274 ate here that Delta7 mice, a model of severe SMA, treated with a suboptimal dose of an SMN2 splicing
278 (unidirectional) constraint on a subordinate SMA-BG loop that determines response-selection, resultin
281 risk predictors were (1) R1 <1.0 mm for the SMA-margin in specific subgroups (upfront pancreatectomy
282 erm trend of surface PM concentration in the SMA declined in the 2000s, but since 2012 its concentrat
283 tion regulation, although only change in the SMA over time occurred in veterans with PTSD and not tho
284 otec, E-ventus BX((R))) was implanted in the SMA, covering the aneurysmal neck and overlapping the pr
285 uppress the self-aggregation tendency of the SMA, thus yielding a polymer:SMA blend with reasonably s
288 a, it can be reasonably anticipated that the SMA treatment landscape will transform significantly.
290 cy may thus be a new therapeutic approach to SMA.SIGNIFICANCE STATEMENT Spinal muscular atrophy (SMA)
291 N deficiency in astrocytes may contribute to SMA is unclear and what aspect of astrocyte activity is
292 xon skipping is the principal contributor to SMA severity, mechanisms governing stability of survival
295 ea of the terminal-group in these A-D-A-type SMAs is a promising approach not only for enhancing the
296 e I SMA patients and most of the widely used SMA mouse models, however, as effective therapeutics are
297 enty-nine children with CM, 77 children with SMA, and 83 CC who presented to Mulago Hospital, Kampala
299 aningful clinical benefits for patients with SMA and, given its mode of action, might be used in comb
300 t with this, mice receiving DCs treated with SMAs exhibited significantly reduced severity of collage
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