Summary

Neurodegeneration refers to the progressive loss of neuron structure or function, which may eventually lead to cell death. Many neurodegenerative diseases, such as amyotrophic lateral sclerosis, multiple sclerosis, Parkinson's disease, Alzheimer's disease, Huntington's disease and prion disease, are the results of neurodegenerative processes. Neurodegeneration can be found in many different levels of neuronal circuits in the brain, from molecules to systems. Since there is no known method to reverse the progressive degeneration of neurons, these diseases are considered incurable. Biomedical research has revealed many similarities between these diseases at the subcellular level, including atypical protein assembly (such as protein diseases) and induction of cell death. These similarities indicate that progress in the treatment of one neurodegenerative disease may also improve other diseases. This collection of entries aims to collect various medical research results related to neurodegeneration. We invite researchers to share their new results and ideas related to neurodegeneration.

Expand All
Entries
Topic Review
Pin1 involvement in Vascular Diseases
The vascular endothelium, the active inner layer of the blood vessel, releases a wide array of biologically active molecules acting in an autocrine or paracrine fashion, thereby controlling arterial structure and vasodilatory, thrombolytic, and vaso-protective functions. By controlling the change of the backbones of several cellular substrates, the peptidyl-prolyl cis-trans isomerase Pin1 acts as key fine-tuner and amplifier of multiple signaling pathways, thereby inducing several biological consequences, both in physiological and pathological conditions. Data from the literature indicate a prominent role of Pin1 in regulating vascular homeostasis. 
  • 466
  • 08 Dec 2021
Topic Review
Parkin Downregulation in TDP-43 Proteinopathies
Parkin and PINK1 are key regulators of mitophagy, an autophagic pathway for selective elimination of dysfunctional mitochondria. To this date, parkin depletion has been associated with recessive early onset Parkinson’s disease (PD) caused by loss-of-function mutations in the PARK2 gene, while, in sporadic PD, the activity and abundance of this protein can be compromised by stress-related modifications. Intriguingly, research in recent years has shown that parkin depletion is not limited to PD but is also observed in other neurodegenerative diseases—especially those characterized by TDP-43 proteinopathies, such as amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). 
  • 434
  • 07 Dec 2021
Topic Review
Rare Genetic Diseases
Rare diseases are conditions that affect a small proportion of the population (fewer than 200,000 persons in the USA or fewer than one in 2000 in Europe). The Orphanet portal for rare diseases and orphan drugs (http://www.orpha.net, accessed on 15 September 2021) currently lists more than 5800 rare diseases. Many are genetically inherited and the genetic causes are clearly identified. From the beginning of the human PSC history, rare genetic disorders have been attractive models for proof-of-concept studies of disease modelling. hESC derived from embryos after pre-implantation genetic diagnosis were a first source of Human pluripotent stem cells (PSCs) with natural, disease-inducing mutations.
  • 466
  • 07 Dec 2021
Topic Review
Autophagy in Human Diseases
Autophagy, a process of cellular self-digestion, delivers intracellular components including superfluous and dysfunctional proteins and organelles to the lysosome for degradation and recycling and is important to maintain cellular homeostasis. In recent decades, autophagy has been found to help fight against a variety of human diseases, but, at the same time, autophagy can also promote the procession of certain pathologies, which makes the connection between autophagy and diseases complex but interesting.
  • 569
  • 06 Dec 2021
Topic Review
Gene Therapy for Mitochondrial Dysfunction in Parkinson’s Disease
Mitochondrial dysfunction has been identified as a pathophysiological hallmark of disease onset and progression in patients with Parkinsonian disorders. Besides the overall emergence of gene therapies in treating these patients, this highly relevant molecular concept has not yet been defined as a target for gene therapeutic approaches. 
  • 654
  • 03 Dec 2021
Topic Review
Aducanumab for Low- and Middle-Income Countries
Aducanumab is a human monoclonal antibody that works to reduce Aβ load in the brain; it is the first disease-modifying therapy to be approved for AD treatment. Current AD treatment is centered on supportive care to manage the debilitating symptoms of dementia, and pharmacotherapy goals of mainstay classes of drugs, such as cholinesterase inhibitors (ChEIs) and N-methyl-D-aspartate (NMDA) receptor antagonists, do not modify the course of the disease. A substantial proportion of patients with Alzheimer’s disease live in low- and middle-income countries (LMICs), and the debilitating effects of this disease exerts burdens on patients and caregivers in addition to the significant economic strains many nations bear.
  • 619
  • 22 Mar 2022
Topic Review
Transthyretin
Transthyretin (TTR) is a thyroid hormone-binding protein which transports thyroxine from the bloodstream to the brain. The structural stability of TTR in tetrameric form is crucial for maintaining its original functions in blood or cerebrospinal fluid (CSF). The altered structure of TTR due to genetic mutations or its deposits due to aggregation could cause several deadly diseases such as cardiomyopathy and neuropathy in autonomic, motor, and sensory systems.
  • 1.1K
  • 03 Dec 2021
Topic Review
Oxidative Stress and Beta Amyloid in Alzheimer’s Disease
The pathogenesis of Alzheimer's disease (AD) involves beta amyloid accumulation known to induce synaptic dysfunction and neurodegenration. The brain's vulnerability to oxidative stress (OS) is considered a crucial detrimental factor in AD. OS and beta amyloid are linked each other because beta amyloid induces OS and, in turn, OS induces beta amyloid accumulation. Evidence indicates that a gradual oxidative damage  accumulation precedes and results in the appearance of pathological AD symptoms. Moreover, OS plays a crucial role in the pathogenesis of many risk factors for AD.
  • 552
  • 02 Dec 2021
Topic Review
Physiological Function of Alpha-Synuclein
Synucleinopathy underlies a wide spectrum of clinical syndromes, including Parkinson's disease (PD), Parkinson's disease dementia (PDD), dementia with Lewy bodies (DLB), multiple system atrophy (MSA), and pure autonomic failure (PAF). A common feature of these syndromes is alpha-synuclein (aS) aggregation and cellular inclusions.  In synucleinopathies, the formation of the distinct αS species is determined by the nature of the self-assembly processes, which is influenced by many factors including the SNCA mutation or multiplication, epigenetic regulation, post-translational modification, micro-environments, etc.  Both the oligomeric and fibrillar forms of αS are toxic to cells.  The detrimental effects of αS continue to grow as αS fibrils start to form LBs, which can cause mitochondrial disassembly, mitophagy, mitochondrial depolarization, and synaptic dysfunction that result in progressive neurodegeneration.  
  • 714
  • 16 Sep 2023
Topic Review
Neuroglobin in Retinal Neurodegeneration
Retinal neurodegeneration affects an increasing number of people worldwide causing vision impairments and blindness, reducing quality of life, and generating a great economic challenge. Due to the complexity of the tissue, and the diversity of retinal neurodegenerative diseases in terms of etiology and clinical presentation, so far, there are no cures. The discovering of the intracellular monomeric globin neuroglobin (NGB), found at high concentration in the retina, has opened new possibilities for the treatment of retinal disease.
  • 478
  • 30 Nov 2021
  • Page
  • of
  • 49

Featured Entry Collections

>>