• undergo apoptosis
  • All cells will undergo apoptosis in the absence of survival signals, usually peptide growth factors secreted by other cells. (bmj.com)
  • 4 The elimination of cell debris occurs in the absence of an inflammatory response, and this quiet, rapid, and efficient removal of apoptotic cells means that apoptosis can be difficult to detect in tissue: although as many as 50% of the cells in a developing organ may undergo apoptosis, less than 1% of cells are apoptotic at any one time. (bmj.com)
  • High levels of cytoplasmic calcium can also cause the cell to undergo apoptosis. (wikipedia.org)
  • genes
  • The 2 genes associated with SMA are SMN1 (survival motor neuron 1) and SMN2, which are adjacent to each other on band 5q. (medscape.com)
  • Isoform 2: Able to act as transcription factor, cannot regulate the expression of the same subset of genes than isoform 1. (uniprot.org)
  • Tumor protein p53, also known as p53, cellular tumor antigen p53 (UniProt name), phosphoprotein p53, tumor suppressor p53, antigen NY-CO-13, or transformation-related protein 53 (TRP53), is any isoform of a protein encoded by homologous genes in various organisms, such as TP53 (humans) and Trp53 (mice). (wikipedia.org)
  • cellular
  • These lipid peroxides can directly damage cells and tissues through interaction with cellular macromolecules, including proteins, lipids, and nucleic acids. (aspetjournals.org)
  • Dysregulation of apoptosis is seen after cellular insults or in neurodegeneration as well as in tumourigenesis. (bmj.com)
  • In contrast, apoptosis is cellular suicide. (bmj.com)
  • Here, we show fibrillarin domain conservation, structure and interacting molecules in different cellular processes as well as with several viral proteins during virus infection. (embl-heidelberg.de)
  • Despite its abundant use as a household protein, little is known about its exact cellular localization and dynamics. (embl-heidelberg.de)
  • Gradually some of the cells stop dividing and differentiate into neurons and glial cells, which are the main cellular components of the CNS. (wikipedia.org)
  • Research by Mattson in the area of molecular and cellular mechanisms that regulate neuronal plasticity and survival, in the contexts of brain development and aging, has established a new conceptual framework for understanding the pathogenesis of neurodegenerative disorders. (wikipedia.org)
  • In particular, he has been a leader in advancing an understanding of the molecular events that destabilize cellular calcium homeostasis and ultimately cause the death of neurons in Alzheimer's disease, Parkinson's disease, ALS and stroke. (wikipedia.org)
  • signal transduction
  • Specifically, we determined the effect of HNE on the activities of mitogen-activated protein (MAP) kinases involved in early signal transduction. (aspetjournals.org)
  • These diseases cause progressive deterioration of the neuron resulting in decreased signal transduction and in some cases even neuronal death. (wikipedia.org)
  • Calcium can act in signal transduction resulting from activation of ion channels or as a second messenger caused by indirect signal transduction pathways such as G protein-coupled receptors. (wikipedia.org)
  • mRNA
  • Exposure of dopaminergic-like cell line to Ibogaine HCl results in an increase in GDNF mRNA, leading to protein expression and to the corresponding activation of the GDNF signaling pathway. (wikipedia.org)
  • These proteins are associated with pre-mRNAs in the nucleus and appear to influence pre-mRNA processing and other aspects of mRNA metabolism and transport. (wikipedia.org)
  • SMN1
  • The SMN1 protein has been associated with the assembly of spliceosomal ribonucleoproteins, which are critical to messenger RNA processing. (medscape.com)
  • developmental
  • Programmed cell death by apoptosis occurs in many developmental processes, including body sculpting (such as digit formation), elimination of self-reacting immune cells, sexual organ development and gamete formation. (bmj.com)
  • Exerts its major developmental effects in somatosensory neurons and in brainstem nuclei involved in motor control. (uniprot.org)
  • axons
  • Peripheral branches of sensory axons fasciculate with the motor axons that extend toward the peripheral muscles from the central nervous system (CNS), whereas the central branches of proprioceptive sensory neurons directly innervate motoneurons. (biologists.org)
  • For example, proprioceptive sensory neurons extend axons to alpha-motoneurons in the ventral horn, and provide information about muscle length and tension to the spinal cord. (biologists.org)
  • neuromuscular
  • Evidence shows that SMN is also involved in the stabilization and maturation of the neuromuscular junction and the transportation of axonal mRNAs in motor neurons [ 25 - 27 ]. (hindawi.com)
  • On the other hand, inappropriate apoptosis resulting in the removal of healthy cells can occur in diseases such as infection, hypoxic-ischaemic injury, neurodegenerative or neuromuscular diseases, and AIDS. (bmj.com)
  • vitro
  • In vitro pulldown assays showed that SMN interacts with H3K79me1,2 at its functional Tudor domain. (embl-heidelberg.de)
  • molecular
  • Although anatomically well documented, the molecular mechanism underlying sensory-motor interaction during neural circuit formation is not fully understood. (biologists.org)
  • In this study, we investigated the molecular mechanism of HNE-induced apoptosis in these cells. (aspetjournals.org)
  • Fibrillarin is an essential protein that is well known as a molecular marker of transcriptionally active RNA polymerase I. Fibrillarin methyltransferase activity is the primary known source of methylation for more than 100 methylated sites involved in the first steps of preribosomal processing and required for structural ribosome stability. (embl-heidelberg.de)
  • The molecular motor kinesin drives anterograde transport from the cell body outward along the axon. (jneurosci.org)
  • binds
  • Chromatin immunoprecipitation confirmed that SMN binds to H3K79me1,2-containing chromatin in iCDR-induced cells. (embl-heidelberg.de)
  • NGF is released from the target cells, binds to and activates its high affinity receptor TrkA on the neuron, and is internalized into the responsive neuron. (wikipedia.org)
  • DAG recruits protein kinase C (PKC), attaching it to the plasma membrane IP3 diffuses to the endoplasmic reticulum, and binds to an IP3 receptor, The IP3 receptor serves as a Ca2+ channel, and releases Ca2+ from the endoplasmic reticulum. (wikipedia.org)
  • Many of Ca2+-mediated events occur when the released Ca2+ binds to and activates the regulatory protein calmodulin. (wikipedia.org)
  • hippocampal
  • Pubmed ID: 11835755 To explore the correlation between cognition disorder and morphologic change of hippocampal neurons after traumatic brain injury (TBI). (jove.com)
  • pathways
  • However, pronounced changes in dynein-associated cargo from survival-promoting to death-promoting signaling pathways are observed as an early event in neurodegeneration. (jneurosci.org)
  • Panobinostat inhibits multiple histone deacetylase enzymes, a mechanism leading to apoptosis of malignant cells via multiple pathways. (wikipedia.org)
  • Thus, neurotrophic signaling may trigger apoptosis rather than survival pathways in cells expressing the p75 receptor in the absence of Trk receptors. (wikipedia.org)
  • Dr. Mattson established signaling pathways by which neurons respond adaptively to bioenergetic and environmental challenges in ways that bolster neuroplasticity and resistance to neurodegenerative disorders. (wikipedia.org)
  • receptor
  • Binding of GDNF and neurturin to human GDNF family receptor alpha 1 and 2. (wikipedia.org)
  • The glucocorticoid receptor is associated with the RNA-binding nuclear matrix protein hnRNP U". J. Biol. (wikipedia.org)
  • Through a protein signaling cascade requiring Erk, CaM KII/IV, PI3K, and PLC, NMDA receptor activation is capable of triggering BDNF exon IV transcription. (wikipedia.org)
  • Many cell surface receptors, including G protein-coupled receptors and receptor tyrosine kinases activate the phospholipase C (PLC) enzyme. (wikipedia.org)
  • onset
  • ALS is a rapidly progressive disorder, typically causing death within 2-5 years of onset ( Pasinelli and Brown, 2006 ). (frontiersin.org)
  • Levels of reactive oxygen species were increased in motor neurons from ALS mice compared with wild-type mice at age 10 weeks, before symptom onset. (aspetjournals.org)
  • The faster aggregating proteins were associated with shorter survival after disease onset. (alzforum.org)
  • Might proteins that are inherently more unstable and more likely to unfold aggregate faster and cause even more rapid onset? (alzforum.org)
  • Hence, a shift from survival-promoting to death-promoting retrograde signaling may be key to the rapid onset of neurodegeneration seen in ALS. (jneurosci.org)
  • mice
  • Our study also suggests that modulation of caspase activity may provide protective benefit in the treatment of ALS, a view that is consistent with our recent demonstration of caspase inhibition extending the survival of transgenic mSOD1 mice. (jneurosci.org)
  • In the present study, we demonstrate (1) that caspase-1 and caspase-3 are activated sequentially in the spinal cords of affected transgenic mSOD1 mice, (2) that activated caspase-3 is localized within neurons of the anterior horn that exhibit apoptotic features, and (3) that overexpression of Bcl-2 delays caspase activation in these animals. (jneurosci.org)
  • Stem cell transplantation in SMA model mice resulted in improvement of motor behaviors and extension of survival, likely from trophic support. (hindawi.com)
  • Knockout mice also exhibit cerebellar abnormalities and an increase in the number of sympathetic neurons. (wikipedia.org)
  • apoptotic
  • Overexpression of the anti-apoptotic protein Bcl-2 attenuates neurodegeneration and delays activation of the caspases and fragmentation of β-actin. (jneurosci.org)
  • Bcl-2 homology-3 domain (BH3)-only proteins are pro-apoptotic members of the Bcl-2 family whose enhanced expression acts as a trigger for the intrinsic apoptotic cascade. (frontiersin.org)
  • The upregulation of a specific subset of BH3-only proteins, including Bid, DP5/Hrk, and BNip3L, in reactive astrocytes suggests that these proteins may execute a novel non-apoptotic function within astrocytes to promote ALS disease progression, thus providing a new potential target for therapeutic intervention. (frontiersin.org)
  • activation domain 2 (AD2) important for apoptotic activity: residues 43-63. (wikipedia.org)
  • precursors
  • SMN serves as a chaperone in the assembly of spliceosome precursors by combining small nuclear RNA (snRNA) molecules with Sm proteins to generate small nuclear ribonucleoproteins (snRNPs) [ 22 , 23 ]. (hindawi.com)
  • Rats
  • Study on Cognition Disorder and Morphologic Change of Neurons in Hippocampus Area Following Traumatic Brain Injury in Rats Chinese Journal of Traumatology = Zhonghua Chuang Shang Za Zhi. (jove.com)
  • Grafting these cells into different parts of rats generates motor neurons regardless of the transplanted cells' microenvironment. (wikipedia.org)
  • nervous system
  • These two brain structures are responsible for motor functions and linking the nervous system to the endocrine system, respectively. (wikipedia.org)
  • Peripheral nervous system (PNS) diseases may be further categorized by the type of nerve cell (motor, sensory, or both) affected by the disorder. (wikipedia.org)
  • During the development of the vertebrate nervous system, many neurons become redundant (because they have died, failed to connect to target cells, etc.) and are eliminated. (wikipedia.org)
  • They are small proteins that secrete into the nervous system to help keep nerve cells alive. (wikipedia.org)
  • ligand
  • The mature form of the protein is a ligand for the product of the RET (rearranged during transfection) protooncogene. (wikipedia.org)
  • differentiate
  • The type of growth factor signaling can effect whether or not NSCs differentiate into motor neurons or not. (wikipedia.org)
  • Directly inhibiting PI3K in NSCs leads to a population of cells that are purely HB9+ and differentiate at an elevated efficiency into motor neurons. (wikipedia.org)
  • They belong to a class of growth factors, secreted proteins that are capable of signaling particular cells to survive, differentiate, or grow. (wikipedia.org)
  • autosomal
  • SMA exhibits an autosomal recessive pattern of inheritance with an incidence of 1 in 6,000-10,000 newborns and a carrier frequency of about 1 : 35 [ 2 , 3 ]. (hindawi.com)
  • SMN2
  • [ 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 the protein produced. (medscape.com)
  • concomitant
  • Cleavage occurs at the SALD site, resulting in a loss of DNA-binding activity and a concomitant detachment of this protein from nuclear structural sites. (wikipedia.org)