New multiplex real-time PCR approach to detect gene mutations for spinal muscular atrophy | BMC Neurology | Full Text
... apoptosis inhibitory protein (NAIP) and general transcription factor IIH, polypeptide 2 gene (GTF2H2) was established and ... The multiple real-time PCR for detecting the mutations of survival of motor neuron (SMN), ... The multiple real-time PCR for detecting the mutations of survival of motor neuron (SMN), apoptosis inhibitory protein (NAIP) ... Genetic circuitry of Survival motor neuron, the gene underlying spinal muscular atrophy. Proc Natl Acad Sci U S A. 2013;110(26 ...https://0-bmcneurol-biomedcentral-com.brum.beds.ac.uk/articles/10.1186/s12883-016-0651-y
RSC96 Schwann Cell Proliferation and Survival Induced by Dilong through PI3K/Akt Signaling Mediated by IGF-I
This hormone protects neurons in the peripheral nervous system from apoptosis by activating the PI3K/Akt pathway, which in turn ... The protein expression of cell cycle regulatory proteins cyclin A, cyclin D1 and cyclin E were determined by western blot. - ... IGF-I acts as a therapeutic target for the treatment of peripheral nerve injury and motor neuron diseases . ... In mammals, central neurons without myelin sheaths are very difficult to regenerate. In contrast, the axons of neurons in the ...https://www.hindawi.com/journals/ecam/2011/216148/
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
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://medicpdf.com/c/cardiosleep.fr1.html
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
AdipoGen Life Sciences
Adipogen offers also Services for Biochemical (Small Molecule), Antibody and Protein Production, ELISA Kit custom sample ... anti-Survival Motor Neuron Protein (human), mAb (7B10). UNIQUE Glycylated Tubulin Antibody anti-Tubulin (glycylated), pAb (Gly- ... MegaFasL - Potent Apoptosis Inducer FasL (human) (multimeric) (rec.). Potent & Specific H+-ATPase Inhibitors High Purity ... Asprosin - A New Fasting-induced Protein NEW Asprosin-specific Abs. Unique UBL Protein-Based Substrates Rhodamine-labeled ISG15 ...https://adipogen.com/
Kugelberg Welander Spinal Muscular Atrophy: Background, Pathophysiology, Epidemiology
Genotype-phenotype correlation of survival motor neuron and neuronal apoptosis inhibitory protein genes in spinal muscular ... Loss of these cells results in a progressive lower motor neuron disease that has no sensory involvement and that is manifested ... They also found histologic evidence of loss of motor neurons in the anterior horn cells of this region. Hoffmann called the ... The motor nuclei in the lower brainstem, usually those of cranial nerves V-XII (V, VII, IX, XII), also may be involved. Various ...https://emedicine.medscape.com/article/306812-overview
Spinal Muscle Atrophy: Background, Pathophysiology, Etiology
The characteristic muscle weakness occurs because of a progressive degeneration of the alpha motor neuron from anterior horn ... Survival motor neuron protein in the nucleolus of mammalian neurons. Brain Res. 2002 Aug 2. 945 (2):160-73. [Medline]. ... This gene is normally inactive during the fetal period and allows normal apoptosis in the developing fetus. The gene becomes ... SMA is caused by a mutation in the survival motor neuron (SMN) gene. ...https://emedicine.medscape.com/article/1264401-overview
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
Motoneuron apoptosis is blocked by CEP-1347 (KT 7515), a novel inhibitor of the JNK signaling pathway. - Semantic Scholar
... because CEP-1347 did not inhibit p38-regulated mitogen-activated protein kinase-activated protein kinase-2 (MAPKAP2) activity ... The IC50 of CEP-1347 for JNK1 activation was the same as the EC50 for motoneuron survival. Inhibition of JNK1 activation by CEP ... This is the first demonstration of a small organic molecule that promotes motoneuron survival and that simultaneously inhibits ... We identified a molecule, CEP-1347 (KT7515), that rescues motoneurons undergoing apoptosis and investigated its effect on ERK1 ...https://www.semanticscholar.org/paper/Motoneuron-apoptosis-is-blocked-by-CEP-1347-%28KT-a-Maroney-Glicksman/a6f2ca10243bc413cd3d57c525bb79fb9b38e9f0
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
Motor Neuron Survival following Motor Axotomy in Newborn Rats. The ability of TRO19622 to promote motor neuron survival or ... Endogenous bax translocation in SH-SY5Y human neuroblastoma cells and cerebellar granule neurons undergoing apoptosis. J ... Use of an exchange assay to determine their photointeraction with the rat uterine estrogen binding protein. Biochemistry 13: ... Automated Measurement of Motor Neuron Survival and Neurite Outgrowth. Motor neuron cultures were stained with calcein-AM, and ...http://jpet.aspetjournals.org/content/322/2/709
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
Survival of motor neuron-related-splicing factor 30
Overexpression causes apoptosis. This gene is a paralog of SMN1 gene, which encodes the survival motor neuron protein, ... HCA RNA Cell Line for Survival of motor neuron-related-splicing factor 30. ... Compartment GO Terms for Survival of motor neuron-related-splicing factor 30. ... The protein encoded by this gene is a nuclear protein that has been identified as a constituent of the spliceosome complex. ...https://pharos.nih.gov/idg/targets/O75940
Selected statins produce rapid spinal motor neuron loss in vitro | BMC Musculoskeletal Disorders | Full Text
We examined the effects of statins on cultured neurons and Schwann cells. Cultured spinal motor neurons were grown on transwell ... 7 days of exposure to fluvastatin depleted spinal motor neurons in a dose-dependent manner with a KD of < 2 μM. Profound ... In contrast, no such toxicity was observed for cultured Schwann cells or cortical neurons. It is known from pharmacokinetic ... Cultured cortical neurons and Schwann cells were assessed using dynamic viability markers. ...https://bmcmusculoskeletdisord.biomedcentral.com/articles/10.1186/1471-2474-13-100
Mice lacking the beta3 subunit of the GABAA receptor have the epilepsy phenotype and many of the behavioral characteristics of...
At least one other gene, the E6-associated protein ubiquitin-protein ligase (UBE3A) gene, has been implicated in AS, so the ... Additionally, these mice exhibited learning and memory deficits, poor motor skills on a repetitive task, hyperactivity, and a ... deletions of neuronal apoptosis inhibitor protein and survival motor neuron genes.. *Martin J. Somerville, Alasdair G. W. ... A deficit of functional GABA(A) receptors in neurons of beta 3 subunit knockout mice.. *Matthew D. Krasowski, Catherine Rick, ...https://www.semanticscholar.org/paper/Mice-lacking-the-beta3-subunit-of-the-GABAA-have-of-Delorey-Handforth/85c970a986050d1245d9da2001fdbe4b65480290
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/
Recombinant Rat CNTF (08P98383) - MP Biomedicals
CNTF is also important for the survival of other neural cell types including primary sensory neurons, motor neurons, basal ... Proteins, Enzymes & Peptides Radiochemicals Rare & Fine Chemicals >> View All Life Sciences Research. Apoptosis Cell Biology ... Bovine Proteins. Bovine Albumin Bovine Transferrin CellMaxx Thrombin >> View All Bovine Proteins Empowered. Amino Acids and ... CNTF is a potent neural factor that was originally characterized as a vital factor for the survival of chick ciliary neurons in ...https://www.mpbio.com/product.php?pid=08P98383&country=223
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?id=1887
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
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