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.

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Entries
Topic Review
Exosomes in Motor Neurone Disease
Exosomes are attractive as vehicle systems for small therapeutic molecules and/or biomolecules including nucleic acids and proteins because of their lipid nature, presence of specific surface ligands (CD11b and CD18 receptors, integrins, tetraspanins) and ability to cross the blood–brain barrier. When compared to other drug delivery systems, exosomes have the distinct advantages of blood–brain barrier penetrance, longer duration in systemic circulation, tissue specificity that minimizes unwanted toxicity or off-target effects, stability of content, desirable biocompatibility and minimal toxicity issues. Techniques such as fusion expression, exosome membrane surface display and anchoring platforms have been used to attach peptides and biological ligands of interest to adhesion molecules, tetraspanins or integrins on exosome surface to ensure targeted delivery and enhanced uptake into desired cells.
  • 794
  • 08 Nov 2021
Topic Review
Misregulation of Wnt Signaling Pathways
Wnt signaling pathways constitute a group of signal transduction pathways that direct many physiological processes, such as development, growth, and differentiation. Dysregulation of these pathways is thus associated with many pathological processes, including neurodegenerative diseases, metabolic disorders, and cancer. At the same time, alterations are observed in plasma membrane compositions, lipid organizations, and ordered membrane domains in brain and metabolic diseases that are associated with Wnt signaling pathway activation.
  • 1.1K
  • 03 Nov 2021
Topic Review
Metabolomics in Retinal Diseases
The retina is abundant with highly specialized neurons that receive, process, and transduce light signals. It is composed of the monolayered retinal pigment epithelium (RPE) and the multi-layered neural retina, which contains five major types of neurons and is regarded as part of the central nervous system.
  • 552
  • 19 Nov 2021
Topic Review
Graphene-Based Biosensors to Detect Dopamine
Parkinson’s disease (PD) is a neurodegenerative disease in which the neurotransmitter dopamine (DA) depletes due to the progressive loss of nigrostriatal neurons. Therefore, DA measurement might be a useful diagnostic tool for targeting the early stages of PD, as well as helping to optimize DA replacement therapy. Moreover, DA sensing appears to be a useful analytical tool in complex biological systems in PD studies. Graphene-based DA sensors are emerging analytical tools for PD diagnostics.
  • 482
  • 04 Nov 2021
Topic Review
Medium-Chain Length Fatty Acids Enhance Aβ Degradation
The accumulation of amyloid β-protein (Aβ) is one of the major pathological hallmarks of Alzheimer’s disease. Insulin-degrading enzyme (IDE), a zinc-metalloprotease, is a key enzyme involved in Aβ degradation, which, in addition to Aβ production, is critical for Aβ homeostasis. Here, we demonstrate that saturated medium-chain fatty acids (MCFAs) increase total Aβ degradation whereas longer saturated fatty acids result in an inhibition of its degradation, an effect which could not be detected in IDE knock-down cells. Further analysis of the underlying molecular mechanism revealed that MCFAs result in an increased exosomal IDE secretion, leading to an elevated extracellular and a decreased intracellular IDE level whereas gene expression of IDE was unaffected in dependence of the chain length. Additionally, MCFAs directly elevated the enzyme activity of recombinant IDE, while longer-chain length fatty acids resulted in an inhibited IDE activity. The effect of MCFAs on IDE activity could be confirmed in mice fed with a MCFA-enriched diet, revealing an increased IDE activity in serum. Our data underline that not only polyunsaturated fatty acids such as docosahexaenoic acid (DHA), but also short-chain fatty acids, highly enriched, for example in coconut oil, might be beneficial in preventing or treating Alzheimer’s disease.
  • 354
  • 03 Nov 2021
Topic Review
Spinal Muscular Atrophy Diagnosis and Dried Saliva Spots
Spinal muscular atrophy (SMA) is a lower motor neuron disease, once considered incurable. The main symptoms are muscle weakness and muscular atrophy. More than 90% of cases of SMA are caused by homozygous deletion of survival motor neuron 1 (SMN1). Emerging treatments, such as splicing modulation of SMN2 and SMN gene replacement therapy, have improved the prognoses and motor functions of patients. However, confirmed diagnosis by SMN1 testing is often delayed, suggesting the presence of diagnosis-delayed or undiagnosed cases. To enable patients to access the right treatments, a screening system for SMA is essential. Even so, the current newborn screening system using dried blood spots is still invasive and cumbersome. Here, we developed a completely non-invasive screening system using dried saliva spots (DSS) as an alternative DNA source to detect SMN1 deletion. In this study, 60 DSS (40 SMA patients and 20 controls) were tested. The combination of modified competitive oligonucleotide priming-polymerase chain reaction and melting peak analysis clearly distinguished DSS samples with and without SMN1. In conclusion, these results suggest that our system with DSS is applicable to SMA patient detection in the real world.
  • 468
  • 29 Oct 2021
Topic Review
Macrophage Migration Inhibitory Factor in Alzheimer′s Disease
The most common form of dementia is accounted for by Alzheimer′s disease (AD) that includes between 50% and 75% of the cases of dementia with a doubling of its prevalence every five years after the age of 65 years. Macrophage migration inhibitory factor (MIF) is a pleiotropic cytokine produced by several cells of the innate and adaptive immune system, as well as non-immune cells. Dismantling the exact role of MIF and its receptors in AD may offer novel diagnostic and therapeutic opportunities in AD. 
  • 367
  • 29 Oct 2021
Topic Review
Ocular Neurodegenerative Diseases
It is well known the connection between the eye and the brain due to the optic nerve, so that, the retina is considered the window of the brain. Therefore the interconnection between neurodegenerative ocular diseases [i.e. glaucoma, Age-related Macular Degeneration (AMD), diabetic retinopathy, retinitis pigmentosa] and neurodegenerative pathologies of the central nervous system (CNS) (i.e. Alzheimer's disease, Parkinson's disease) are also defined.
  • 592
  • 01 Nov 2021
Topic Review
Ferulic Acid in Animal Models of Alzheimer’s Disease
Alzheimer’s disease (AD) is a neurodegenerative disease with a high incidence in the elderly. Many preclinical studies show that a natural product, ferulic acid (FA), displays neuroprotective effects in AD models. 
  • 574
  • 27 Oct 2021
Topic Review
Diabetic Retinopathy Treatment
Diabetic retinopathy (DR) is one of the leading matters of vision-loss globally. Even though there have been extensive efforts to reduce vision loss, the prevalence of DR is still uprising. There are several pharmacological and surgical therapies currently used in clinics, but none of them has not been used as effective therapies at the early stage of DR.
  • 581
  • 01 Nov 2021
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