Differentiation apoptosis survival of motor neuron 2 protein. Medical search. Frequent questions

The recombinant form of this protein was shown to promote the survival and differentiation of dopaminergic neurons in culture, and was able to prevent apoptosis of motor neurons induced by axotomy. (wikipedia.org)
PD is mainly recognized when first symptoms of motor parkinsonism (hypokinesia, bradykinesia, rigidity, tremor and the loss of postural reflexes) develop as the result of the loss of the majority of the dopaminergic neurons of the pars compacta of the substantia nigra with a striatal dopaminergic depletion of over 80% [ 6 ]. (biomedcentral.com)
This study optimized conditions for the differentiation of hONPs towards dopaminergic neurons. (scirp.org)
Several neurotrophic factors were detected in the pre-transfected hONPs which have potential roles in the maintenance, survival and proliferation of dopaminergic neurons. (scirp.org)

The tumor necrosis factor-like weak inducer of apoptosis (TWEAK)/TNF receptor superfamily member fibroblast growth factor inducible 14 (Fn14) pathway has been shown to play a critical role in the regulation of denervation-induced muscle atrophy as well as muscle proliferation, differentiation and metabolism in adults. (keele.ac.uk)
Like CNTF, it promotes the survival of embryonic motor neurons, and could increase the proliferation of neural precursor cells in the presence of EGF and FGF-2. (peprotech.com)

In addition, BDNF shows the trophic effects on the sensory neurons of the nodose ganglia. (fujifilm.com)
Patients affected by SCAN-1 develop an adult onset devastating pathology characterized by peripheral axonal motor, sensory neuropathy, distal muscular atrophy, pes cavus and steppage gait. (ataxia.org)
In a survey of trafficking regulators required for developmental dendrite pruning in Drosophila sensory neurons, the small GTPase Rab11 was identified as a regulator of recycling endosomes. (sdbonline.org)
However, whereas a direct role for Sox10 activity has been established in pigment and glial lineages, this is more controversial in NC-derived sensory neurons of the dorsal root ganglia (DRGs). (silverchair.com)
We proposed that sox10 functioned in specification of sensory neurons, whereas others suggested that sensory neuronal defects were merely secondary to absence of glia. (silverchair.com)
Here we provide evidence that in zebrafish,early DRG sensory neuron survival is independent of differentiated glia. (silverchair.com)
Critically, we demonstrate that Sox10 is expressed transiently in the sensory neuron lineage, and specifies sensory neuron precursors by regulating the proneural gene neurogenin1 . (silverchair.com)

Background: Spinal muscular atrophy (SMA) is a childhood neuromuscular disorder caused by depletion of the survival motor neuron (SMN) protein. (keele.ac.uk)
SMA is characterized by the selective death of spinal cord motor neurons, leading to progressive muscle wasting. (keele.ac.uk)
Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease characterized by the degeneration of motor neurons in the brain and spinal cord. (nature.com)
Very interestingly, a vital insight of neuroinflammation research in ALS was generated by the evidence that both the mRNA and protein levels of the pro-inflammatory enzyme cyclooxygenase-2 (COX-2) are upregulated in both transgenic mouse models and in human postmortem brain and spinal cord. (nature.com)
Spinal muscular atrophy (SMA) is a neurodegenerative disease produced by low levels of Survival Motor Neuron (SMN) protein that affects alpha motoneurons in the spinal cord. (mdpi.com)
This therapeutic effect is multifactorial, including the regulation of the activity of various endogenic biological mediators, the regeneration of nerve fibres and stem cells and the inhibition of inflammation, neural apoptosis and oxidative stress in the injured spinal cord. (eexot-journal.com)
2. Masri R, Keller A. Chronic pain following spinal cord injury. (eexot-journal.com)
Histological analyses of the lumbosacral spinal cord revealed that Bxt markedly delayed the early motor-neuron degeneration occurring at presymptomatic stages in ALS-transgenic mice. (frontiersin.org)
A series of pathological changes after spinal cord injury (SCI) are usually associated with oxidative stress, inflammation, and apoptosis. (bvsalud.org)
AAVrg's were injected to deliver cre recombinase and/or a red fluorescent protein (RFP) under the human Synapsin 1 promoter (hSyn1) into the spinal cords of C57BL/6 PTENFloxΔ/Δ mice to knockout PTEN (PTEN-KO) in a severe thoracic SCI crush model at both acute and chronic time points. (bvsalud.org)
BDA labeling from the motor cortex revealed a dense corticospinal tract (CST) bundle in all groups except chronically treated PTEN-KO mice, indicating a potential long-term toxic effect of PTEN-KO to neurons in the motor cortex which was corroborated by a loss of ß-tubulin III labeling above the lesion within spinal cords after PTEN-KO. (bvsalud.org)

GDNF has the ability to activate the ERK-1 and ERK-2 isoforms of MAP kinase in sympathetic neurons as well as P13K/AKT pathways via activation of its receptor tyrosine kinases. (wikipedia.org)
While NGF acts on a portion of the sympathetic neurons of dorsal root ganglia, BDNF acts on neurons of them that do not overlap with each other. (fujifilm.com)

The decreased progenitor cell pool indicates that Fgf2 is required for the development of a subset of cortical progenitors within the dorsal PVE, which in turn may differentiate into cortical projection neurons. (jneurosci.org)
Because the dorsal PVE was depleted of progenitors but the ganglionic eminences were not affected in Fgf2 −/− mice, we predicted that these mice should lack a population of pyramidal neurons, but their cortical GABA interneurons should have remained unchanged. (jneurosci.org)

At the molecular level, Cul3 regulates cytoskeletal and adhesion protein abundance in mouse embryos. (nature.com)
The major intracellular signaling systems are common to receptor-type tyrosine kinases and include the 1) the PI3K-Akt system, which regulates apoptosis and translation, 2) the Ras-MAPK system, which induces differentiation mainly through transcriptional regulation, and 3) the PLCγ-Ca2+ system, which drives intracellular calcium signaling (5). (fujifilm.com)
The GTPase Rab11 regulates endosomal protein trafficking and previous work has shown that loss of Rab11 caused intestinal inflammation and hyperplasia in mice and flies. (sdbonline.org)

It may start with a range of non-motor symptoms such as disorders of the autonomic nervous system, olfaction, sleep, mood and subtle cognitive deterioration, before a degeneration of the dopamine producing cells in the upper brainstem (nigral substance) may manifest with motor parkinsonism, the clinical hallmark of this disease, and way before involvement of the neocortex induces dementia [ 5 ]. (biomedcentral.com)
Loss of Pcs impairs Rab11 localization on the trans-side of Golgi units and induces the cytoplasmic accumulation of post-Golgi vesicles bearing rhabdomere proteins, as observed in Rab11-deficiency. (sdbonline.org)

In my laboratory researchers are examining the roles played by axonal cytoskeletal proteins in nervous system development, and in the etiology of Alzheimer's and other related neurodegenerative diseases. (uml.edu)
It functions as a molecular chaperone, aiding the refolding of non-native proteins, and plays a critical role in stabilisation of the cytoskeleton through interactions with several cytoskeletal components, such as actin, intermediate filaments and microtubules [ 7 ]. (biomedcentral.com)

This secondary damage includes oxidative stress and inflammatory cascades that can lead to further neuronal loss and motor deficits. (researchsquare.com)

The role of HSP27 in the pathogenesis of neurodegenerative disorders such as frontotemporal lobar degeneration (FTLD), Alzheimer's disease (AD) and motor neuron disease (MND) was investigated. (biomedcentral.com)

The proteolytic processing of GDNF requires SorLA, a protein sorting receptor. (wikipedia.org)
This molecule binds to all neurotrophins with a low affinity (Kd=10 -9 M), belongs to the tumor necrosis factor receptor family, is involved in apoptosis, and is again attracting attention as a receptor on which pro-neurotrophins act. (fujifilm.com)
Using this knowledge we characterized genetic strategies to label developing γ-MNs based on GDNF receptor expression, showed their strict dependence for survival on muscle spindle-derived GDNF and generated an animal model in which γ-MNs are selectively lost. (biomedcentral.com)

Neurons, with their distinct neurites, require elaborate membrane trafficking pathways and regulation to uphold neurite identity and to be able to respond to neuronal or developmental stimuli. (sdbonline.org)
The accumulation of misfolded proteins may adversely affect neuronal connectivity and plasticity and trigger cell death signalling pathways [ 2 ]. (biomedcentral.com)

E3 ubiquitin ligases regulate cellular protein composition by providing target recognition and specificity to the ubiquitin-dependent proteasomal degradation pathway 12 . (nature.com)
CUL3 is a conserved protein of the Cullin family, comprising eight members, which contain a conserved cullin homology domain, named after its ability to select cellular proteins for degradation. (nature.com)
Despite the well-understood process of CUL3-mediated protein ubiquitination and degradation 12 , its target proteins in the developing central nervous system and its role in brain development remain utterly understudied. (nature.com)
Rab11 often links vesicles to molecular motors, and this study finds that loss of the microtubule motor dynein also leads to defective Neuroglian and Ppk26 degradation. (sdbonline.org)
It may represent an accumulation of misfolded, damaged or unwanted proteins, awaiting or undergoing degradation. (biomedcentral.com)

Neurotrophic factor is a general term for proteins that maintain neuronal survival, induce differentiation (e.g., induction of neurite outgrowth and expression of neurotransmitter-synthesizing enzymes), promote maturation, and regulate synaptic functions. (fujifilm.com)
Gamma motor neurons (γ-MNs) selectively innervate muscle spindle intrafusal fibers and regulate their sensitivity to stretch. (biomedcentral.com)
Different classes of RNA granules share some protein components and may use similar mechanisms to regulate mRNA translation/decay. (rupress.org)

GDNF is synthesized as a 211 amino acid-long protein precursor, pro-GDNF. (wikipedia.org)
While secretion takes place, the protein precursor folds via a sulfide-sulfide bond and dimerizes. (wikipedia.org)
Finally, the protein precursor undergoes proteolysis due to a proteolytic consensus sequence in its C-terminus end and is cleaved to 134 amino acids. (wikipedia.org)
Because multiple proteases can cleave the protein precursor, four different mature forms of GDNF can be produced. (wikipedia.org)
BDNF cDNA encodes a 247 amino acid residue precursor protein with a signal peptide and a proprotein that are cleaved to yield the 119 amino acid residue mature BDNF. (novusbio.com)

It can be secreted by astrocytes, oligodendrocytes, Schwann cells, motor neurons, and skeletal muscle during the development and growth of neurons and other peripheral cells. (wikipedia.org)
Defective muscle regeneration can severely affect re-innervation by motor axons, and neuromuscular junctions (NMJs) development, ultimately leading to skeletal muscle atrophy. (biomed.news)

The present study unravels a new molecular system for vesicle-based axonal transport of proteins in male and female flies (Drosophila melanogaster). (sdbonline.org)

The effects of neurotrophins on the peripheral nervous system are absolute and essential for survival and neurite extension. (fujifilm.com)
Brain-derived neurotrophic factor (BDNF) is a member of the NGF family of neurotrophic factors (also named neurotrophins) that are required for the differentiation and survival of specific neuronal subpopulations in both the central as well as the peripheral nervous system. (novusbio.com)

In this study, we show that depletion of TDP-43 in microglia, but not in astrocytes, strikingly upregulates cyclooxygenase-2 (COX-2) expression and prostaglandin E2 (PGE2) production through the activation of MAPK/ERK signaling and initiates neurotoxicity. (nature.com)

Because Fgf2 −/− mice lacked 45% of cortical neurons at birth, the progenitor loss is compensated only in part during neurogenesis. (jneurosci.org)
Cultured oligodendrocyte progenitor cells (OPCs) were incubated with HD ± BMSC-derived conditional medium (BMSC-CM). OPC differentiation was studied by immunostaining and morphometric analysis. (biomedcentral.com)

In contrast, loss of the specific subunits of TRAPPII, another known Rab11-GEF, does not cause any defects on the eye development nor the transport of rhabdomere proteins, however, simultaneous loss of TRAPPII and Pcs shows severe defects on eye development. (sdbonline.org)

Research on brain-derived neurotrophic factor (BDNF) has evolved from its "classical" actions of promoting neuronal survival and differentiation to rather novel actions of regulating neurotransmission and synaptic plasticity. (fujifilm.com)
Hence, the effects of neurotrophins on neuronal survival and neurite outgrowth are also referred to as "classical effects" of neurotrophic factors. (fujifilm.com)

Physiol Rev. 2018;98(2):881-917. (eexot-journal.com)

citation needed] It prevents apoptosis in motor neurons during development, decreases the overall loss of neurons during development, rescues cells from axotomy-induced death, and prevents chronic degeneration. (wikipedia.org)
However, the role of microglia in TDP-43-mediated motor neuron degeneration remains poorly understood. (nature.com)

In addition to the transcript encoding GDNF, two additional alternative transcripts encoding distinct proteins, referred to as astrocyte-derived trophic factors, have also been described. (wikipedia.org)
CUL3 ASD-associated genetic variants are most often de novo missense or loss of function (loF) mutations, dispersed throughout the entire gene and affecting distinct protein domains. (nature.com)
The mechanisms that control the differentiation of functionally distinct fusimotor neurons are unknown. (biomedcentral.com)
Investigation of the mechanisms that control γ- from α-MN differentiation has been limited by the lack of available molecular markers to distinguish these functionally distinct subpopulations during development, as molecular differences between postnatal α- and γ-MNs have only recently been demonstrated [ 6 ]. (biomedcentral.com)

We report here that the Fgf2 null mutant mice show an ∼40% decrease in cortical glutamatergic pyramidal neurons. (jneurosci.org)
In contrast to pyramidal neurons, cortical GABA interneurons are unaffected by the lack of Fgf2. (jneurosci.org)

In the central dogma RNA acts as a simple intermediary between the DNA that carries the genetic information and the proteins that define the whole variety of biological processes in the cell. (biomedcentral.com)

Many neurodegenerative diseases are characterised by accumulations of misfolded proteins that can colocalise with chaperone proteins (for example, heat shock protein 27 (HSP27)), which might act as modulators of protein aggregation. (biomedcentral.com)
Many neurodegenerative diseases, also termed as protein conformational diseases [ 1 ], are characterised by accumulations of misfolded proteins that often share morphological and biochemical features and can colocalise with several other proteins, including various chaperone proteins. (biomedcentral.com)

Retrograde tracing with Fluorogold revealed viable neurons throughout the motor cortex despite a loss of RFP expression at 9 weeks post-PTEN-KO. (bvsalud.org)
However, few Fluorogold labeled neurons were detected within the motor cortex at 6 months post-treatment. (bvsalud.org)

Congenital hearing impairment is a common birth defect worldwide, occurring in approximately 1-2 per 1000 infants. (hindawi.com)
2010) Deletion of the WD40 domain of LRRK2 in Zebrafish causes Parkinsonism-like loss of neurons and locomotive defect. (scirp.org)

NGF affects only on specific neurons, suggesting the existence of other molecules with similar functions. (fujifilm.com)
Subsequently, NGF was cloned in 1983 and BDNF in 1989 (2), and the two molecules exhibited more than 50% homology. (fujifilm.com)
A single E.coli bacterium contains ~2 million LPS molecules (2 - 20 fg/cell). (transcriptionfactor.org)

Synaptic Signals from Glutamate-Treated Neurons Induce Aberrant Post-Synaptic Signals in Untreated Neuronal Networks. (uml.edu)

Myosin XVA is a large actin-based motor protein. (hindawi.com)

Hair cells (HCs) in the cochlea mainly function in converting the sound mechanical waves into the electric neural signals [ 2 - 4 ] which make it extremely critical for the hearing ability. (hindawi.com)

The TMC1 gene is located on chromosome 9q21 and contains 24 exons that encodes a 760 amino acid membrane protein TMC1 with six transmembrane domains [ 12 , 13 ]. (hindawi.com)
The MYO15A gene is located at chromosome 17p11.2 and contains 66 coding exons, which encodes an unconventional myosin protein Myosin XVA [ 19 ]. (hindawi.com)

GDNF is a small protein that potently promotes the survival of many types of neurons. (wikipedia.org)

Glial cell line-derived neurotrophic factor (GDNF) is a protein that, in humans, is encoded by the GDNF gene. (wikipedia.org)
Spinocerebellar ataxia type 35 (SCA35) is a rare, autosomal dominant neurodegenerative disorder associated with mutations in TGM6 gene that encode the protein transglutaminase 6 (TG6). (ataxia.org)
This focuses on the expression of the human tdp1 mutant gene in brain tissue of flies lacking the endogenous Gkt protein. (ataxia.org)
Cytoplasmic RNA granules in germ cells (polar and germinal granules), somatic cells (stress granules and processing bodies), and neurons (neuronal granules) have emerged as important players in the posttranscriptional regulation of gene expression. (rupress.org)

Based on findings in neurological diseases ( 20-23 ) and pharmacological interactions ( 24-27 ), it appears that a malfunction of 5-hydroxytryptamine (HT)2 receptors may have a major role in the pathogenesis of bruxism. (spandidos-publications.com)

Recombinant Murine Neuropoietin is a 19.8 kDa protein containing 183 amino acid residues. (peprotech.com)

We found that a single injection of retrogradely transported adeno-associated viruses (AAVrg) to knockout the phosphatase and tensin homolog protein (PTEN) in chronic SCI can effectively target both damaged and spared axons and transiently restore locomotor functions in near-complete injury models. (bvsalud.org)

In vitro cultured OPCs showed more differentiation after incubation with BMSC-CM. Hes1 expression was found to be significantly increased by HD and decreased by BMSC or BMSC-CM. The change of Hes1 was found, however, independent of Notch1 activation, but dependent on TNFα/RelB signaling. (biomedcentral.com)

Survival, weight, and neuromuscular function studies together with histological and biochemical analyses were performed. (frontiersin.org)

Stability of Imprinting and Differentiation Capacity in Naïve Human Cells Induced by Chemical Inhibition of CDK8 and CDK19. (axonmedchem.com)

The pre-sequence leads the protein to the endoplasmic reticulum for secretion. (wikipedia.org)

We also found that postnatal γ-MNs are also distinguished by low expression of the neuronal nuclear protein (NeuN). (biomedcentral.com)
Thus, GCGs contain proteins involved in translation initiation, translation control, and mRNA decay, which is consistent with their proposed role in the regulation ofs maternal mRNA expression. (rupress.org)

Actual therapy only symptomatically affects motor parkinsonism, though. (biomedcentral.com)

Bxt significantly delayed motor function deterioration, ameliorated the loss of body weight, and extended mice survival up to 30% of the symptomatic period. (frontiersin.org)
MYO15A mutations are responsible for congenital deafness DFNB3 in human and cochleovestibular dysfunction in shaker 2 mice which shows abnormally short stereocilia bundles and diminished staircase [ 20 , 21 ]. (hindawi.com)
For these studies we use neurons that are maintained in cell culture as well as genetically-modified strains of mice that are at increased risk for developing neurodegenerative conditions analogous to those observed in humans. (uml.edu)
In addition, motor function in mice was significantly improved after SeNPs-Met-MVs treatment. (bvsalud.org)
Following contusion SCI, wild-type (WT) mice showed significant tissue damage, white matter damage and impaired motor recovery. (researchsquare.com)
However, mice lacking Hv1 ( Hv1 -/- ) showed significant white matter sparing and improved motor recovery. (researchsquare.com)
The improved motor recovery in Hv1 -/- mice was associated with decreased interleukin-1β, reactive oxygen/ nitrogen species production and reduced neuronal loss. (researchsquare.com)

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive weakness and muscle atrophy related to the loss of upper and lower motor neurons (MNs) without a curative treatment. (frontiersin.org)

100% sequence homology with Mouse, Rat, Canine, Equine and all other mammalian proteins examined. (novusbio.com)

From development to aging and disease, the brain parenchyma is under the constant threat of debris accumulation, in the form of dead cells and protein aggregates. (frontiersin.org)

HGF was cloned as a growth factor for hepatocytes ( 1 , 2 ), is identical to scatter factor (SF) and was originally discovered as a fibroblast-derived cell motility factor for epithelial cells ( 3 ). (spandidos-publications.com)
In conclusion, we have found that using AAVrg's to knockout PTEN is an effective manipulation capable of restoring motor functions in chronic SCI and can enhance axon growth of currently unidentified axon populations when delivered acutely after injury. (bvsalud.org)

however, for the large majority of patients lacking access to advanced therapies stroke mortality and residual morbidity remain high and many patients become incapacitated by motor and cognitive deficits, with loss of independence in activities of daily living. (mdpi.com)
It is one of the most common causes of ADNSHL in Mideast countries due to prevalent consanguineous marriage [ 22 , 23 ], with majority associated with prelingual severe-to-profound hearing loss and mutations in exon 2 leading to a milder auditory phenotype [ 23 ]. (hindawi.com)
We previously showed that the toxicity is both linked to a loss and a gain of function of the mutant protein but the neuronal role of the TG6 is still not known. (ataxia.org)
To test the idea that loss of Rab11 may promote cancer progression, archival human patient tissues were analyzed and 51 out of 70 colon cancer tissues had lower Rab11 protein staining. (sdbonline.org)

Taken together, our results reveal a previously unrecognized non-cell-autonomous mechanism in TDP-43-mediated neurodegeneration, identifying COX-2-PGE2 as the molecular events of microglia- but not astrocyte-initiated neurotoxicity and identifying celecoxib as a novel potential therapy for TDP-43-linked ALS and possibly other types of ALS. (nature.com)
GCNA is a histone binding protein required for spermatogonial stem cell maintenance. (axonmedchem.com)
Hexon and penton structures form the capsid of AdV, and fiber protein mediates the binding of the virion to the cell surface and is a major determinant of viral tropism. (genemedi.com)

These data highlight the importance of membrane protein recycling during development. (sdbonline.org)

Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors. (novusbio.com)
These molecular markers can be used to identify γ-MNs from birth to the adult and to distinguish γ- from β-motor axons in the periphery. (biomedcentral.com)

Anatomy62

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Psychiatry and Psychology5

Phenomena and Processes55

Disciplines and Occupations2

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20181

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