Abstract:
:Myotonic dystrophy type 1 (DM1 or Steinert's disease) and type 2 (DM2) are multisystem disorders of genetic origin. Progressive muscular weakness, atrophy and myotonia are the most prominent neuromuscular features of these diseases, while other clinical manifestations such as cardiomyopathy, insulin resistance and cataracts are also common. From a clinical perspective, most DM symptoms are interpreted as a result of an accelerated aging (cataracts, muscular weakness and atrophy, cognitive decline, metabolic dysfunction, etc.), including an increased risk of developing tumors. From this point of view, DM1 could be described as a progeroid syndrome since a notable age-dependent dysfunction of all systems occurs. The underlying molecular disorder in DM1 consists of the existence of a pathological (CTG) triplet expansion in the 3' untranslated region (UTR) of the Dystrophia Myotonica Protein Kinase (DMPK) gene, whereas (CCTG)n repeats in the first intron of the Cellular Nucleic acid Binding Protein/Zinc Finger Protein 9 (CNBP/ZNF9) gene cause DM2. The expansions are transcribed into (CUG)n and (CCUG)n-containing RNA, respectively, which form secondary structures and sequester RNA-binding proteins, such as the splicing factor muscleblind-like protein (MBNL), forming nuclear aggregates known as foci. Other splicing factors, such as CUGBP, are also disrupted, leading to a spliceopathy of a large number of downstream genes linked to the clinical features of these diseases. Skeletal muscle regeneration relies on muscle progenitor cells, known as satellite cells, which are activated after muscle damage, and which proliferate and differentiate to muscle cells, thus regenerating the damaged tissue. Satellite cell dysfunction seems to be a common feature of both age-dependent muscle degeneration (sarcopenia) and muscle wasting in DM and other muscle degenerative diseases. This review aims to describe the cellular, molecular and macrostructural processes involved in the muscular degeneration seen in DM patients, highlighting the similarities found with muscle aging.
journal_name
Front Aging Neuroscijournal_title
Frontiers in aging neuroscienceauthors
Mateos-Aierdi AJ,Goicoechea M,Aiastui A,Fernández-Torrón R,Garcia-Puga M,Matheu A,López de Munain Adoi
10.3389/fnagi.2015.00125subject
Has Abstractpub_date
2015-07-09 00:00:00pages
125issn
1663-4365journal_volume
7pub_type
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journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2013.00023
更新日期:2013-06-24 00:00:00
abstract::Similar to humans, the normal aged rat population is not homogeneous in terms of cognitive function. Two distinct subpopulations of aged Sprague-Dawley rats can be identified on the basis of spatial memory performance in the hole-board paradigm. It was the aim of the study to reveal protein changes relevant to aging a...
journal_title:Frontiers in aging neuroscience
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journal_title:Frontiers in aging neuroscience
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journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2019.00047
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abstract::Alzheimer's disease (AD) is marked by the presence of amyloid beta (Aβ) plaques, neurofibrillary tangles (NFT), neuronal death and synaptic loss, and inflammation in the brain. AD research has, in large part, been dedicated to the understanding of Aβ and NFT deposition as well as to the pharmacological reduction of th...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章,评审
doi:10.3389/fnagi.2019.00337
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abstract::The globus pallidus occupies a critical position in the indirect pathway of the basal ganglia motor control system. Hyperpolarization-activated cyclic-nucleotide gated (HCN) channels play an important role in the modulation of neuronal excitability. In vivo extracellular single unit recording, behavioral test and immu...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2019.00190
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abstract::Understanding speech in the presence of background sound can be challenging for older adults. Speech comprehension in noise appears to depend on working memory and executive-control processes (e.g., Heald and Nusbaum, 2014), and their augmentation through training may have rehabilitative potential for age-related hear...
journal_title:Frontiers in aging neuroscience
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doi:10.3389/fnagi.2016.00049
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journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2010.00026
更新日期:2010-08-06 00:00:00
abstract::Symptoms in late-onset neuromuscular disorders initiate only from midlife onward and progress with age. These disorders are primarily determined by identified hereditable mutations, but their late-onset symptom manifestation is not fully understood. Here, we review recent research developments on the late-onset autoso...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章,评审
doi:10.3389/fnagi.2014.00317
更新日期:2014-11-10 00:00:00
abstract::Cardiorespiratory fitness has been shown to protect and enhance cognitive and brain functions, but little is known about the cortical mechanisms that underlie these changes in older adults. In this study, functional near infrared spectroscopy (fNIRS) was used to investigate variations in oxyhemoglobin [HbO2] and in de...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2014.00272
更新日期:2014-10-08 00:00:00
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journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2016.00035
更新日期:2016-02-29 00:00:00
abstract::[This corrects the article on p. 13 in vol. 8, PMID: 26903857.]. ...
journal_title:Frontiers in aging neuroscience
pub_type: 已发布勘误
doi:10.3389/fnagi.2017.00035
更新日期:2017-02-23 00:00:00
abstract::Background: Mild cognitive impairment (MCI) is the early phase of Alzheimer's disease (AD). The aim of early intervention for MCI is to decrease the rate of conversion from MCI to AD. However, the efficacy of multiple interventions in MCI, and the optimal methods of delivery, remain controversial. We aimed to compare ...
journal_title:Frontiers in aging neuroscience
pub_type:
doi:10.3389/fnagi.2020.00121
更新日期:2020-06-05 00:00:00
abstract::The semantic verbal fluency task is broadly used in the neuropsychological assessment of elderly subjects. Even some studies have identified differences in verbal fluency clustering and switching measures between subjects with normal aging and a clinical condition such as mild cognitive impairment (MCI) and Alzheimer'...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2014.00141
更新日期:2014-07-01 00:00:00
abstract::Most therapeutic agents are designed to target a molecule or pathway without consideration of the mechanisms involved in the physiological turnover or removal of that target. In light of this and in particular for Alzheimer's disease, a number of therapeutic interventions are presently being developed/investigated whi...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章,评审
doi:10.3389/fnagi.2015.00064
更新日期:2015-05-05 00:00:00
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journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2018.00313
更新日期:2018-10-09 00:00:00
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journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2016.00272
更新日期:2016-11-22 00:00:00
abstract::During aging, human response times (RTs) to unisensory and crossmodal stimuli decrease. However, the elderly benefit more from crossmodal stimulus representations than younger people. The underlying short-latency multisensory integration process is mediated by direct crossmodal connections at the level of primary sens...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2018.00052
更新日期:2018-03-02 00:00:00
abstract::Background: Gray matter (GM) alterations in Parkinson's disease (PD) patients with rapid eye movement sleep behavior disorder (RBD) have been demonstrated in many neuroimaging studies using voxel-based morphometry (VBM). However, the inconsistent findings between studies cannot be applied to clinical practice as a neu...
journal_title:Frontiers in aging neuroscience
pub_type:
doi:10.3389/fnagi.2020.00213
更新日期:2020-08-12 00:00:00
abstract::Background and Objective: Ventricle volume is closely related to hydrocephalus, brain atrophy, Alzheimer's, Parkinson's syndrome, and other diseases. To accurately measure the volume of the ventricles for elderly patients, we use deep learning to establish a systematic and comprehensive automated ventricle segmentatio...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2020.618538
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journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2015.00148
更新日期:2015-07-29 00:00:00
abstract::Oxidative stress (OS) contributes to Alzheimer's disease (AD) pathology. OS can be a result of increased reactive oxygen/nitrogen species, reduced antioxidants, oxidatively damaged molecules, and/or a combination of these factors. Scientific literature is scarce for the markers of OS-specific for detecting AD at an ea...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章,评审
doi:10.3389/fnagi.2020.00231
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abstract::Objective: Cerebral small vessel disease (SVD) is associated with increased mortality, disability and cognitive decline, depression in stroke survivors. This study examined the association between SVD burden, defined by a combination of SVD markers, and health-related quality of life (HRQoL) in acute ischemic stroke. ...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2017.00372
更新日期:2017-11-13 00:00:00
abstract::Parkinson's disease (PD) shows high heterogeneity with regard to the underlying molecular pathogenesis involving multiple pathways and mechanisms. Diagnosis is still challenging and rests entirely on clinical features. Thus, there is an urgent need for robust diagnostic biofluid markers. Untargeted metabolomics allows...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2018.00051
更新日期:2018-03-05 00:00:00
abstract::Inter-temporal choice depends on multiple, interacting systems, some of which may be compromised with age. Some of these systems may be responsible for ongoing trial-by-trial choice strategies. Some may represent the consequences of action. Some may be necessary for the coupling between anticipated consequences and st...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2015.00041
更新日期:2015-03-27 00:00:00
abstract::Parkinson's disease (PD) is the second most prevalent neurodegenerative disease in ageing individuals. It is now clear that genetic susceptibility and environmental factors play a role in disease etiology and progression. Because environmental factors are involved with the majority of the cases of PD, it is important ...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章,评审
doi:10.3389/fnagi.2014.00036
更新日期:2014-03-07 00:00:00
abstract::Alzheimer's disease (AD) is a neurodegenerative disorder associated with the progressive dysfunction of cognitive ability. Previous research has indicated that the default mode network (DMN) is closely related to cognition and is impaired in Alzheimer's disease. Because recent studies have shown that different frequen...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2017.00259
更新日期:2017-08-03 00:00:00
abstract::Hypertension has a profound influence on the structure and function of blood vessels. Cerebral vessels undergo both structural and functional changes in hypertensive animals. However, dynamic changes of cerebrovasculature and the factors involved in this process are largely unknown. In this study, we explored the dyna...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2017.00359
更新日期:2017-11-02 00:00:00
abstract::Objectives: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common hereditary small vessel disease, with reported frequencies of 2-5/100,000 individuals. Recently, it has been reported that some patients with NOTCH3 gene mutations show atypical clinical ...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2020.00130
更新日期:2020-05-14 00:00:00
abstract::Background: Alzheimer's disease (AD) and Parkinson's disease (PD) are two major neurodegenerative diseases worldwide. Demographic aging is in rapid progress in China. Up-to-date estimates of AD and PD prevalence have not been provided. Methods: Studies that reported the prevalence of AD and PD in China were identified...
journal_title:Frontiers in aging neuroscience
pub_type: 杂志文章
doi:10.3389/fnagi.2020.603854
更新日期:2020-12-21 00:00:00