Browse by Subjects
Neuronal Degeneration
Submit
Sort:
Show:
Page Size:
Topic review
Updated time: 22 Apr 2021
ALS
Submitted by: Andrew Eisen
Definition: The site of origin of amyotrophic lateral sclerosis (ALS), although unsettled, is increasingly recognized as being cortico-fugal, which is a dying-forward process primarily starting in the corticomotoneuronal system.
Entry Collection : Neuronal Degeneration
Unfold
Topic review
Updated time: 10 May 2021
Submitted by: Adolfo Toledano
Definition: Classical scrapie is a naturally transmissible spongiform neurodegenerative disease that originally affected sheep, goats and mouflons and that has been observed for several centuries. It is a “strange” disease that cannot be classified as a classical bacterial or viral infectious disease and does not follow the pathogenic patterns identified by infectious or neurodegenerative disease research.
Entry Collection : Neuronal Degeneration
Unfold
Topic review
Updated time: 15 Mar 2021
Submitted by: Maurits Vissers
Definition: The clinical failure rate for disease-modifying treatments (DMTs) that slow or stop disease progression has been nearly 100% for the major neurodegenerative disorders (NDDs), with many compounds failing in expensive and time-consuming phase 2 and 3 trials for lack of efficacy. However, as our understanding of NDDs is improving, there is a rise in potentially disease-modifying treatments being brought to the clinic. Further increasing the rational use of mechanistic biomarkers in early phase trials for these (targeted) therapies can increase R&D productivity with a quick win/fast fail approach in an area that has seen a nearly 100% failure rate to date.
Entry Collection : Neuronal Degeneration
Unfold
Topic review
Updated time: 05 Mar 2021
Submitted by: Kimitoshi Nakamura
Definition: Dissociated optic nerve fiber layer (DONFL) appearance is characterized by dimpling of the fundus when observed after vitrectomy with the internal limiting membrane (ILM) peeling in macular diseases. However, the cause of DONFL remains largely unknown. Optical coherence tomography (OCT) findings have indicated that the nerve fiber layer (NFL) and ganglion cells are likely to have been damaged in patients with DONFL appearance. Since DONFL appearance occurs at a certain postoperative period, it is unlikely to be retinal damage directly caused by ILM peeling because apoptosis occurs at a certain period after tissue damage and/or injury. However, it may be due to ILM peeling-induced apoptosis in the retinal tissue. Anoikis is a type of apoptosis that occurs in anchorage-dependent cells upon detachment of those cells from the surrounding extracellular matrix (i.e., the loss of cell anchorage). The anoikis-related proteins βA3/A1 crystallin and E-cadherin are reportedly expressed in retinal ganglion cells.
Entry Collection : Neuronal Degeneration
Unfold
Topic review
Updated time: 13 May 2021
Submitted by: Koji Aoyama
Definition: Glutathione (GSH) is the most abundant non-protein thiol, and plays crucial roles in the antioxidant defense system and the maintenance of redox homeostasis in neurons.
Entry Collection : Neuronal Degeneration
Unfold
Topic review
Updated time: 07 May 2021
Submitted by: Basavaraj S. Balapal
Definition: Advances achieved with molecular biology and genomics technologies have permitted investigators to discover epigenetic mechanisms, such as DNA methylation and histone posttranslational modifications, which are critical for gene expression in almost all tissues and in brain health and disease. These advances have influenced much interest in understanding the dysregulation of epigenetic mechanisms in neurodegenerative disorders. Although these disorders diverge in their fundamental causes and pathophysiology, several involve the dysregulation of histone methylation-mediated gene expression. Interestingly, epigenetic remodeling via histone methylation in specific brain regions has been suggested to play a critical function in the neurobiology of psychiatric disorders, including that related to neurodegenerative diseases. Prominently, epigenetic dysregulation currently brings considerable interest as an essential player in neurodegenerative disorders, such as Alzheimer’s disease (AD), Parkinson’s disease (PD), Huntington’s disease (HD), Amyotrophic lateral sclerosis (ALS) and drugs of abuse, including alcohol abuse disorder, where it may facilitate connections between genetic and environmental risk factors or directly influence disease-specific pathological factors.
Entry Collection : Neuronal Degeneration
Unfold
Topic review
Updated time: 03 Mar 2021
Submitted by: Federica Piancone
Definition: Neurodegenerative diseases are chronic, progressive disorders that occur in the central nervous system (CNS). They are characterized by the loss of neuronal structure and function and are associated with inflammation. Inflammation of the CNS is called neuroinflammation, which has been implicated in most neurodegenerative diseases, including Alzheimer’s disease (AD), Parkinson’s disease (PD), amyotrophic lateral sclerosis (ALS) and multiple sclerosis (MS). Much evidence indicates that these different conditions share a common inflammatory mechanism: the activation of the inflammasome complex in peripheral monocytes and in microglia, with the consequent production of high quantities of the pro-inflammatory cytokines IL-1β and IL-18. Inflammasomes are a group of multimeric signaling complexes that include a sensor Nod-like receptor (NLR) molecule, the adaptor protein ASC, and caspase-1. The NLRP3 inflammasome is currently the best-characterized inflammasome. Multiple signals, which are potentially provided in combination and include endogenous danger signals and pathogens, trigger the formation of an active inflammasome, which, in turn, will stimulate the cleavage and the release of bioactive cytokines including IL-1β and IL-18.
Entry Collection : Neuronal Degeneration
Unfold
Topic review
Updated time: 20 Feb 2021
Submitted by: Donald Alcendor
Definition: The JC polyomavirus (JCPyV/JCV) is a member of thePolyomaviridae family and is ubiquitious in the general population, infecting 50–80% of individuals globally. The virus remains latent but can reactivate under conditions of immunosuppression and cause life-threatening disease such as progressive multifocal leukoencephalopathy (PML). PML can be a complication of HIV disease especially in HIV patients who are not receiving anti retroviral therapy. In immunocompetent individuals, PML is rare however, the incidence of PML is increasing due to the widespread use of immune modulating therapies. JCV induced PML is rare in solid organ transplant patients but there is documented cases that correlate with immunosuppression that is required allograft transplantation. Currently there no curative therapies for PML with high mortality after diagnosis.
Entry Collection : Neuronal Degeneration
Unfold
Topic review
Updated time: 12 May 2021
Submitted by: Simona Bungau
Definition: Neurodegenerative disorders/diseases (NDs) are a chronic debilitating group of heterogeneous diseases, which include loss of neuronal function and structure, leading to neuronal cell death or progressive degeneration. NDs comprise a highly complex etiology that is mainly associated with abnormal protein accumulation, mutated genes, increased reactive oxygen species (ROS), neuroinflammation, mitochondrial dysfunction, apoptosis, elevated endoplasmic reticulum (ER), calcium overload, excitotoxicity, or neuronal destruction in specific regions of the brain. ND is a wide array of neurological disorders that generally affect central nervous system (CNS) neurons, characterized by progressive neuronal dysfunction in the CNS, resulting in deficit of specific functions of the brain (movement, memory, and cognition). These processes are involved in the pathogenesis and progression of NDs, such as Huntington’s disease, Alzheimer’s disease, amyotrophic lateral sclerosis, multiple sclerosis, and Parkinson’s disease.
Entry Collection : Neuronal Degeneration
Unfold
Topic review
Updated time: 10 May 2021
Submitted by: Mari Dezawa
Definition: Muse cells, identified as pluripotent surface marker, stage-specific embryonic antigen (SSEA)-3(+), are endogenous reparative pluripotent stem cells distributed in the bone marrow, peripheral blood and connective tissue of every organ. Since they are non-tumorigenic and do not require gene introduction or cytokine treatment to be rendered pluripotent and induce differentiation, they elicit few safety concerns. They can be delivered intravenously and do not require surgery for their administration since they selectively home to damaged site by sphingosine-1-phosphate (S1P)-S1PR2 axis after intravenous injection. Donor-Muse cells can be used without HLA-matching test or immunosuppressant treatment since they have a specific immunomodulatory system represented by HLA-G expression.
Entry Collection : Neuronal Degeneration
Unfold
  • Page
  • of
  • 2