*  KAKEN - Research Projects | Clinical, Epidemiological and Molecular Genetical Research for Spinal Muscular Atrophy (KAKENHI...
SMA is caused by mutation of the survival motor neuron (SMN) gene. We conducted a molecular genetic analysis of SMA patients ... and compared clinical severity with deletions of the SMNt and neuronal apoptosis inhibitory protein (NAIP) genes, and C212 and ... Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder characterized by degeneration of motor neurons ... Spinal muscular atrophy (type 1, type 2)'Jpn J Clin Med. suppl. 364-370 (1999). *. Description. 「研究成果報告書概要(欧文)」より ...
  https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-10670762/
*  Delaying Caspase Activation by Bcl-2: A Clue to Disease Retardation in a Transgenic Mouse Model of Amyotrophic Lateral...
... one cannot extrapolate from their numbers either the number of neurons dying by apoptosis or the number of motor neurons dying ... protein aggregation (Durham et al., 1997), aberrant protein-protein interactions (Kunst et al., 1997), or decreased binding ... 1997) Effects of wild-type and mutated copper/zinc superoxide dismutase on neuronal survival and l-DOPA-induced toxicity in ... For each section, right and left counts of motor neurons were averaged. The averaged number of motor neurons in each L3 section ...
  http://www.jneurosci.org/content/20/24/9119
*  NRN1 - Wikipedia
"Interaction of survival of motor neuron (SMN) and HuD proteins with mRNA cpg15 rescues motor neuron axonal deficits". Proc. ... Gao R, Wang L, Sun J, Nie K, Jian H, Gao L, Liao X, Zhang H, Huang J, Gan S (2014). "MiR-204 promotes apoptosis in oxidative ... The encoded protein contains a consensus cleavage signal found in glycosylphoshatidylinositol (GPI)-anchored proteins. The ... Neuritin 1 is a protein that in humans is encoded by the NRN1 gene. This gene encodes a member of the neuritin family, and is ...
  https://en.wikipedia.org/wiki/NRN1
*  cardiosleep.fr
the survival motor neuron gene (SMN) 1. The patient in Case 2 was a 37-year-old female lacking exons 7 and 8 in SMN 1 and exon ... 5 in the neuronal apoptosis inhibitory protein (NAIP) gene. We applied continuous positive airway pressure (CPAP) in both cases ... sensitivity C-reactive protein (CRP), peptide C,TNF-alpha, IL-6, and IL-10. Patients with hypertension (n = 40) had higher ... high-sensitivity C-reactive protein (OR, 1.48; CI, 1.13-1.95; P=0.005) were significantly associated with patients with IMT,= ...
  http://pharmapdf.com/c/cardiosleep.fr1.html
*  Astrocytes in amyotrophic lateral sclerosis: direct effects on motor neuron survival
binding proteins in motor neurons [26], the mitochondria seem to play a crucial role in buffering Ca2. +. [24, 27]. ... culture media from activated astrocytes from symptomatic mutant SOD1 mice induce apoptosis of purified motor neuron cultures. ... only enhance the motor neuron death of mutant SOD1-containing motor neurons but also induces cell death in normal motor neurons ... Moreover, GRPs extend survival and disease duration, attenuate motor neuron loss, and slow decline in forelimb motor and ...
  http://pubmedcentralcanada.ca/pmcc/articles/PMC2750739/
*  Kugelberg Welander Spinal Muscular Atrophy Clinical Presentation: History, Physical, Causes
Genotype-phenotype correlation of survival motor neuron and neuronal apoptosis inhibitory protein genes in spinal muscular ... The SMN1 protein has also been associated with the NAIP (neuronal apoptosis inhibitory protein) gene, a gene that helps to ... survival motor neuron 1) and SMN2, which are adjacent to each other on band 5q. SMN1 is believed to be the primary disease- ... 10] SMN2 differs from it by 1 C-T transition in exon 7, leading to alternate splicing and a nonfunctional protein for 70-90% of ...
  https://emedicine.medscape.com/article/306812-clinical
*  Frontiers | The Bcl-2 Homology-3 Domain (BH3)-Only Proteins, Bid, DP5/Hrk, and BNip3L, Are Upregulated in Reactive Astrocytes...
Large alpha motor neurons in lumbar spinal cord sections of both WT and end-stage mutant SOD1 mice stained positively for a ... several studies have provided evidence of a central role for intrinsic apoptosis. Bcl-2 homology-3 domain (BH3)-only proteins ... Large alpha motor neurons in lumbar spinal cord sections of both WT and end-stage mutant SOD1 mice stained positively for a ... Finally, examination of protein expression using western blotting also revealed marked increases in DP5/Hrk and BNip3L in G93A ...
  https://www.frontiersin.org/articles/10.3389/fncel.2018.00015/full
*  Role of motoneuron-derived neurotrophin 3 in survival and axonal projection of sensory neurons during neural circuit formation ...
7), it is conceivable that the motoneuron-derived Ntf3 can act as a local signal to prevent apoptosis of sensory neurons. As ... 2002). Common developmental requirement for Olig function indicates a motor neuron/oligodendrocyte connection. Cell 109, 75-86 ... Secreted Ntf3-GFP protein reaches DRG in vivo. (A-C) Fluorescence micrographs of transverse sections of GFP and DsRed- ... Sensory neurons receive motoneuron-derived Ntf3 as a survival factor. At E10 to E12, Ntf3 acts as a pre-target neurotrophin. ...
  http://dev.biologists.org/content/139/6/1125?iss=6
*  Selective Activation of the c-Jun N-Terminal Protein Kinase Pathway during 4-Hydroxynonenal-Induced Apoptosis of PC12 Cells |...
... and withdrawal of survival factors (Le-Niculescu et al., 1999). It is also true that motor neuron apoptosis is blocked by a ... 1998) Motor neuron apoptosis is blocked by CEP-1347 (KT 7515), a novel inhibitor of the JNK signaling pathway. J Neurosci 18: ... stress-activated protein kinase. JNK. c-Jun N-terminal protein kinase. p38 MAP kinase. p38 mitogen-activated protein kinase. ... mitogen-activated protein kinase. MEKK. MAP kinase kinase kinase. ASK1. apoptosis signal-regulating kinase 1. HA. hemagglutinin ...
  http://molpharm.aspetjournals.org/content/58/3/535
*  Concurrent Administration of Neu2000 and Lithium Produces Marked Improvement of Motor Neuron Survival, Motor Function, and...
We examined whether the Fas pathway would mediate apoptosis in ALS mice. Expression of Fas and its cytoplasmic adaptor protein ... apoptosis probably contributes to motor neuron degeneration in ALS. Degenerating spinal cord and motor cortex neurons are ... Concurrent Administration of Neu2000 and Lithium Produces Marked Improvement of Motor Neuron Survival, Motor Function, and ... Concurrent Administration of Neu2000 and Lithium Produces Marked Improvement of Motor Neuron Survival, Motor Function, and ...
  http://molpharm.aspetjournals.org/content/71/4/965
*  Molecular Neurodegeneration | Full text | Endogenous TDP-43, but not FUS, contributes to stress granule assembly via G3BP
Lastly, we observe that SG assembly is a contributing factor in the survival of neuronal-like cells responding to acute ... is an ALS-causative gene that we have previously implicated in the regulation of the core stress granule proteins G3BP and TIA- ... is a fatal neurodegenerative disease characterized by the selective loss of upper and lower motor neurons, a cell type that is ... Hua Y, Zhou J: Survival motor neuron protein facilitates assembly of stress granules. FEBS Lett 2004, 572:69-74. PubMed ...
  http://www.molecularneurodegeneration.com/content/7/1/54
*  Therapy Development for Spinal Muscular Atrophy in SMN Independent Targets
... why do low levels of survival motor neuron protein make motor neurons sick?" Nature Reviews Neuroscience, vol. 10, no. 8, pp. ... motor neurons degenerate in spinal anterior horn regions probably through cell apoptosis [16, 34], and muscle atrophy and motor ... "Interaction of survival of motor neuron (SMN) and HuD proteins with mRNA cpg15 rescues motor neuron axonal deficits," ... C. Fallini, H. Zhang, Y. Su et al., "The Survival of Motor Neuron (SMN) protein interacts with the mRNA-binding protein HuD and ...
  https://www.hindawi.com/journals/np/2012/456478/
*  Apoptosis in neural development and disease | ADC Fetal & Neonatal Edition
Spinal muscular atrophy is associated with mutations in the survival of motor neuron and neuronal apoptosis inhibitory protein ... 59 This implies that mutations in NAIP could deregulate apoptosis in spinal motor neurons, thus causing their death. The ... will undergo apoptosis if deprived of survival factors, suggesting that death not survival is the default fate.22 It follows ... NAIP) genes.57 58 NAIP is related to the baculovirus inhibitor of apoptosis protein and inhibits apoptosis in several cell ...
  http://fn.bmj.com/content/77/3/F165
*  ALS: Predicting Prognosis, Banking on Pluripotent Stem Cells | ALZFORUM
Motor Neurons From ALS Patients. By deriving motor neurons from reprogrammed skin fibroblasts, John Dimos and colleagues have ... Paper: Protein aggregation and protein instability govern familial amyotrophic lateral sclerosis patient survival. ... or downstream effects such as mitochondrial-induced apoptosis (see ARF related news story). ... Currently, there is no way of culturing motor neurons directly from humans, so studying these derived motor neurons in culture ...
  https://www.alzforum.org/news/research-news/als-predicting-prognosis-banking-pluripotent-stem-cells
*  Glial cell line-derived neurotrophic factor - Wikipedia
... and was able to prevent apoptosis of motor neurons induced by axotomy. The encoded protein is processed to a mature secreted ... GDNF is a small protein that potently promotes the survival of many types of neurons. It signals through GFRα receptors, ... The recombinant form of this protein was shown to promote the survival and differentiation of dopaminergic neurons in culture, ... The most prominent feature of GDNF is its ability to support the survival of dopaminergic neurons. He DY, Ron D (2006). " ...
  https://en.wikipedia.org/wiki/Glial_cell_line-derived_neurotrophic_factor
*  Pou4f1 - POU domain, class 4, transcription factor 1 - Rattus norvegicus (Rat) - Pou4f1 gene & protein
Exerts its major developmental effects in somatosensory neurons and in brainstem nuclei involved in motor control. Stimulates ... Regulates the expression of specific genes involved in differentiation and survival within a subset of neuronal lineages ( ... Ativates BCL2 expression and protects neuronal cells from apoptosis (via the N-terminal domain). Induces neuronal process ... Regulates dorsal root ganglion sensory neuron specification and axonal projection into the spinal cord. Plays a role in TNFSF11 ...
  http://www.uniprot.org/uniprot/P20266
*  SMART: Secondary literature for Fibrillarin domain
Mutation of the survival motor neurons 1 (SMN1) gene causes motor neuron apoptosis and represents the major cause of spinal ... Reduction of the survival of motor neurons (SMN) protein levels causes the motor neuron degenerative disease spinal muscular ... survival of motor neurons protein) and consequent loss of motor neurons. SMN is involved in snRNP transport and nuclear RNA ... The survival motor neuron (SMN) protein, the protein product of the spinal muscular atrophy (SMA) disease gene, plays a role in ...
  http://smart.embl-heidelberg.de/smart/show_secondary.cgi?domain=Fibrillarin
*  Protocols and Video Articles Authored by Junling Gao
The increased motor neuron differentiation was accompanied by a decrease in the number of glial fibrillary acidic protein (GFAP ... The therapeutic efficacy of stem cell therapy depends greatly on the survival of grafted stem cell-derived motor neurons in the ... stress-mediated apoptosis. Several studies have demonstrated that ischemia reperfusion results in apoptosis. Oxidative stress ... motor neurons in vitro. More importantly, blocking PI3K not only enhances motor neuron differentiation from hNSCs grafted into ...
  https://www.jove.com/author/Junling_Gao
*  Essay For Chemistry In Our Daily Life writing a good personal statement for psychology graduate school accessibility
Lower incidence of deletions in the survival of motor neuron gene and the neuronal apoptosis inhibitory protein gene in ... Chapter 50 Sensory and Motor Mechanisms 1117 Taste in Mammals Humans and other mammals perceive five tastes: sweet, sour, salty ... 6 Conclusions: Our is Essay most daily RRD, life bilateral in 59 daily group 1 and life of group 2, daily common daily young ... PR: 2 Amherst Real Estate DeWitt Real Estate and Auctions Our, VA has been helping families find our dreams homes chemistry ...
  http://coxamurusizim.c0.pl/termpaper/essay-for-chemistry-in-our-daily-life.php
*  Development of the nervous system - Wikipedia
CNTF: Ciliary neurotrophic factor is another protein that acts as a survival factor for motor neurons. CNTF acts via a receptor ... limiting amounts of target-derived trophic factors and axons that fail to receive sufficient trophic support die by apoptosis. ... The survival of neurons is regulated by survival factors, called trophic factors. The neurotrophic hypothesis was formulated by ... Synaptic communication between neurons leads to the establishment of functional neural circuits that mediate sensory and motor ...
  https://en.wikipedia.org/wiki/Development_of_the_nervous_system
*  A Switch in Retrograde Signaling from Survival to Stress in Rapid-Onset Neurodegeneration | Journal of Neuroscience
2003) Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice. Science 302:113-117, Erratum (2003 ... Many of the proteins that showed a decreased association with dynein in the mSOD1 model were proteins from survival pathways ( ... 2005) Axonal dynactin p150Glued transports caspase-8 to drive retrograde olfactory receptor neuron apoptosis. J Neurosci 25: ... 1993) Neurotrophins promote motor neuron survival and are present in embryonic limb bud. Nature 363:266-270. ...
  http://www.jneurosci.org/content/29/31/9903.full?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=1&andorexacttitle=and&andorexacttitleabs=and&fulltext=kinexus&andorexactfulltext=and&searchid=1&FIRSTINDEX=0&sortspec=relevance&resourcetype=HWCIT
*  Neurotrophic Factors and Neurodegeneration | Springer for Research & Development
... are molecules which act to promote the differentiation of neurons and to maintain their phenotype. The discovery by Hamburger ... Eagleson KL, Bennett MR (1983) Survival of purified motor neurons in vitro: effects of skeletal muscle conditioned medium. ... Halegoua S, Patrick J (1980) Nerve growth factor mediates phosphorylation of specific proteins. Cell 22: 571-581PubMedGoogle ... Muscle apoptosis as a proposed pathogenetic mechanism. Brain 113:433-445Google Scholar ...
  https://rd.springer.com/chapter/10.1007/978-1-4471-1871-8_12
*  Protocols and Video Articles Authored by Michela Pozzobon
Mutations in the survival of motor neuron gene (SMN1) are responsible for spinal muscular atrophy, a fatal neuromuscular ... and enterocyte apoptosis and proliferation. RESULTS: AFS cells integrated in the bowel wall and improved rat survival and ... Except for those treated with hAFS cells, moderate hyperoxia induced an increase in protein content of IL-6, IL-1β, as well as ... The Src family kinase Syk, the B-cell adaptor protein BLNK, and phospholipase C (PLC)-gamma2 were consistently absent from Reed ...
  https://www.jove.com/author/Michela_Pozzobon
*  Microglia and astroglia: the role of neuroinflammation in lead toxicity and neuronal injury in the brain
Mitochondrial dysfunction in astrocytes affects the survival of motor neurons.[44] Accumulation of free radicals and ROS might ... Balbuena P, Li W, Ehrich M. Assessments of tight junction proteins occludin, claudin 5 and scaffold proteins ZO1 and ZO2 in ... impede the supply of energy to neurons, and cause neuronal apoptosis.[14-17] Moreover, recent studies have shown a crucial ... the protein kinase C (PKC)/NF-κB pathway might be involved in lead-induced neuroinflammatory injury to brain neurons, as it ...
  http://nnjournal.net/article/view/1109/564
*  Ro5-4864 - Wikipedia
"Ro5-4864 promotes neonatal motor neuron survival and nerve regeneration in adult rats". The European Journal of Neuroscience. ... Xiao J, Liang D, Zhang H, Liu Y, Li F, Chen YH (April 2010). "4'-Chlorodiazepam, a translocator protein (18 kDa) antagonist, ... regulation of apoptosis and steroidogenesis as part of the host-defense response". Current Pharmaceutical Design. 13 (23): 2385 ... Veenman L, Papadopoulos V, Gavish M (2007). "Channel-like functions of the 18-kDa translocator protein (TSPO): ...
  https://en.wikipedia.org/wiki/Ro5-4864