Articles
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Citation: Molecular Neurodegeneration 2021 16:20
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The CD33 short isoform is a gain-of-function variant that enhances Aβ1–42 phagocytosis in microglia
CD33 is genetically linked to Alzheimer’s disease (AD) susceptibility through differential expression of isoforms in microglia. The role of the human CD33 short isoform (hCD33m), preferentially encoded by an A...
Citation: Molecular Neurodegeneration 2021 16:19 -
Plaque associated microglia hyper-secrete extracellular vesicles and accelerate tau propagation in a humanized APP mouse model
Recent studies suggest that microglia contribute to tau pathology progression in Alzheimer’s disease. Amyloid plaque accumulation transforms microglia, the primary innate immune cells in the brain, into neurod...
Citation: Molecular Neurodegeneration 2021 16:18 -
Preclinical modeling of chronic inhibition of the Parkinson’s disease associated kinase LRRK2 reveals altered function of the endolysosomal system in vivo
The most common mutation in the Leucine-rich repeat kinase 2 gene (LRRK2), G2019S, causes familial Parkinson’s Disease (PD) and renders the encoded protein kinase hyperactive. While targeting LRRK2 activity is...
Citation: Molecular Neurodegeneration 2021 16:17 -
High dose expression of heme oxigenase-1 induces retinal degeneration through ER stress-related DDIT3
Oxidative stress is a common cause of neurodegeneration and plays a central role in retinal degenerative diseases. Heme oxygenase-1 (HMOX1) is a redox-regulated enzyme that is induced in neurodegenerative dise...
Citation: Molecular Neurodegeneration 2021 16:16 -
Cholesterol alters mitophagy by impairing optineurin recruitment and lysosomal clearance in Alzheimer’s disease
Emerging evidence indicates that impaired mitophagy-mediated clearance of defective mitochondria is a critical event in Alzheimer’s disease (AD) pathogenesis. Amyloid-beta (Aβ) metabolism and the microtubule-a...
Citation: Molecular Neurodegeneration 2021 16:15 -
Enhancing GABAergic signaling ameliorates aberrant gamma oscillations of olfactory bulb in AD mouse models
Before the deposition of amyloid-beta plaques and the onset of learning memory deficits, patients with Alzheimer’s disease (AD) experience olfactory dysfunction, typified by a reduced ability to detect, discri...
Citation: Molecular Neurodegeneration 2021 16:14 -
Altered network properties in C9ORF72 repeat expansion cortical neurons are due to synaptic dysfunction
Physiological disturbances in cortical network excitability and plasticity are established and widespread in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) patients, including those harb...
Citation: Molecular Neurodegeneration 2021 16:13 -
Dominant mutations in MIEF1 affect mitochondrial dynamics and cause a singular late onset optic neuropathy
Inherited optic neuropathies are the most common mitochondrial diseases, leading to neurodegeneration involving the irreversible loss of retinal ganglion cells, optic nerve degeneration and central visual loss...
Citation: Molecular Neurodegeneration 2021 16:12 -
Prion protein oligomers cause neuronal cytoskeletal damage in rapidly progressive Alzheimer’s disease
High-density oligomers of the prion protein (HDPs) have previously been identified in brain tissues of patients with rapidly progressive Alzheimer’s disease (rpAD). The current investigation aims at identifyin...
Citation: Molecular Neurodegeneration 2021 16:11 -
Moving fluid biomarkers for Alzheimer’s disease from research tools to routine clinical diagnostics
Four fluid-based biomarkers have been developed into diagnostic tests for Alzheimer’s disease (AD) pathology: the ratio of 42 to 40 amino acid-long amyloid β, a marker of plaque pathology; total-tau and phosph...
Citation: Molecular Neurodegeneration 2021 16:10 -
Deletion of Socs3 in LysM+ cells and Cx3cr1 resulted in age-dependent development of retinal microgliopathy
We generated a mouse model of primary microglial dysfunction by deleting two negative immune regulatory genes, Cx3cr1 and Socs3 (in LysM+ cells). This study aimed to understand how primary microglial dysfunction ...
Citation: Molecular Neurodegeneration 2021 16:9 -
Remodeling Alzheimer-amyloidosis models by seeding
Alzheimer’s disease (AD) is among the most prevalent neurodegenerative diseases, with brain pathology defined by extracellular amyloid beta deposits and intracellular tau aggregates. To aid in research efforts...
Citation: Molecular Neurodegeneration 2021 16:8 -
Increased serum QUIN/KYNA is a reliable biomarker of post-stroke cognitive decline
Strokes are becoming less severe due to increased numbers of intensive care units and improved treatments. As patients survive longer, post-stroke cognitive impairment (PSCI) has become a major health public i...
Citation: Molecular Neurodegeneration 2021 16:7 -
Relationships of gut microbiota, short-chain fatty acids, inflammation, and the gut barrier in Parkinson’s disease
Previous studies have reported that gut microbiota, permeability, short-chain fatty acids (SCFAs), and inflammation are altered in Parkinson’s disease (PD), but how these factors are linked and how they contri...
Citation: Molecular Neurodegeneration 2021 16:6 -
Clusterin secreted from astrocyte promotes excitatory synaptic transmission and ameliorates Alzheimer’s disease neuropathology
Genome-wide association studies have established clusterin (CLU) as a genetic modifier for late-onset Alzheimer’s disease (AD). Both protective and risk alleles have been identified which may be associated wit...
Citation: Molecular Neurodegeneration 2021 16:5 -
Comprehensive metabolic profiling of Parkinson’s disease by liquid chromatography-mass spectrometry
Parkinson’s disease (PD) is a prevalent neurological disease in the elderly with increasing morbidity and mortality. Despite enormous efforts, rapid and accurate diagnosis of PD is still compromised. Metabolom...
Citation: Molecular Neurodegeneration 2021 16:4 -
Methods to investigate intrathecal adaptive immunity in neurodegeneration
Cerebrospinal fluid (CSF) provides basic mechanical and immunological protection to the brain. Historically, analysis of CSF has focused on protein changes, yet recent studies have shed light on cellular alter...
Citation: Molecular Neurodegeneration 2021 16:3 -
New approaches to symptomatic treatments for Alzheimer’s disease
Successful development of agents that improve cognition and behavior in Alzheimer’s disease (AD) is critical to improving the lives of patients manifesting the symptoms of this progressive disorder.
Citation: Molecular Neurodegeneration 2021 16:2 -
Induction of autophagy mitigates TDP-43 pathology and translational repression of neurofilament mRNAs in mouse models of ALS/FTD
TDP-43 proteinopathy is a pathological hallmark of many neurodegenerative diseases including amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). So far, there is no therapy available for the...
Citation: Molecular Neurodegeneration 2021 16:1 -
Cell-autonomous role of Presenilin in age-dependent survival of cortical interneurons
Mutations in the PSEN1 and PSEN2 genes are the major cause of familial Alzheimer’s disease. Previous studies demonstrated that Presenilin (PS), the catalytic subunit of γ-secretase, is required for survival of ex...
Citation: Molecular Neurodegeneration 2020 15:72 -
Astrocyte-derived clusterin suppresses amyloid formation in vivo
Accumulation of amyloid-β (Aβ) peptide in the brain is a pathological hallmark of Alzheimer’s disease (AD). The clusterin (CLU) gene confers a risk for AD and CLU is highly upregulated in AD patients, with the co...
Citation: Molecular Neurodegeneration 2020 15:71 -
Regional binding of tau and amyloid PET tracers in Down syndrome autopsy brain tissue
Tau pathology is a major age-related event in Down syndrome with Alzheimer’s disease (DS-AD). Although recently, several different Tau PET tracers have been developed as biomarkers for AD, these tracers showed...
Citation: Molecular Neurodegeneration 2020 15:68 -
An in vitro bioengineered model of the human arterial neurovascular unit to study neurodegenerative diseases
The neurovascular unit (NVU) – the interaction between the neurons and the cerebrovasculature – is increasingly important to interrogate through human-based experimental models. Although advanced models of cer...
Citation: Molecular Neurodegeneration 2020 15:70 -
Activity of Alzheimer’s γ-secretase is linked to changes of interferon-induced transmembrane proteins (IFITM) in innate immunity
The activity of γ-secretase is critical to the pathogenesis of Alzheimer’s disease (AD). How its activity is regulated is intriguing and highly important for any AD therapy that focuses on reduction of toxic a...
Citation: Molecular Neurodegeneration 2020 15:69 -
A novel systems biology approach to evaluate mouse models of late-onset Alzheimer’s disease
Late-onset Alzheimer’s disease (LOAD) is the most common form of dementia worldwide. To date, animal models of Alzheimer’s have focused on rare familial mutations, due to a lack of frank neuropathology from mo...
Citation: Molecular Neurodegeneration 2020 15:67 -
Diabetic phenotype in mouse and humans reduces the number of microglia around β-amyloid plaques
Alzheimer’s disease (AD) is the most common neurodegenerative disease and type 2 diabetes (T2D) plays an important role in conferring the risk for AD. Although AD and T2D share common features, the common mole...
Citation: Molecular Neurodegeneration 2020 15:66 -
Tauopathy-associated tau modifications selectively impact neurodegeneration and mitophagy in a novel C. elegans single-copy transgenic model
A defining pathological hallmark of the progressive neurodegenerative disorder Alzheimer’s disease (AD) is the accumulation of misfolded tau with abnormal post-translational modifications (PTMs). These include...
Citation: Molecular Neurodegeneration 2020 15:65 -
Conformation-selective tau monoclonal antibodies inhibit tau pathology in primary neurons and a mouse model of Alzheimer’s disease
The spread of tau pathology in Alzheimer’s disease (AD) is mediated by cell-to-cell transmission of pathological tau seeds released from neurons that, upon internalization by recipient neurons, template the mi...
Citation: Molecular Neurodegeneration 2020 15:64 -
APOE2: protective mechanism and therapeutic implications for Alzheimer’s disease
Investigations of apolipoprotein E (APOE) gene, the major genetic risk modifier for Alzheimer’s disease (AD), have yielded significant insights into the pathogenic mechanism. Among the three common coding variant...
Citation: Molecular Neurodegeneration 2020 15:63 -
Trem2 Y38C mutation and loss of Trem2 impairs neuronal synapses in adult mice
Triggering receptor expressed on myeloid cells 2 (TREM2) is expressed in the brain exclusively on microglia and genetic variants are linked to neurodegenerative diseases including Alzheimer’s disease (AD), fro...
Citation: Molecular Neurodegeneration 2020 15:62 -
Aging-relevant human basal forebrain cholinergic neurons as a cell model for Alzheimer’s disease
Alzheimer’s disease (AD) is an adult-onset mental disorder with aging as a major risk factor. Early and progressive degeneration of basal forebrain cholinergic neurons (BFCNs) contributes substantially to cogn...
Citation: Molecular Neurodegeneration 2020 15:61 -
Modeling the β-secretase cleavage site and humanizing amyloid-beta precursor protein in rat and mouse to study Alzheimer’s disease
Three amino acid differences between rodent and human APP affect medically important features, including β-secretase cleavage of APP and Aβ peptide aggregation (De Strooper et al., EMBO J 14:4932-38, 1995; Uen...
Citation: Molecular Neurodegeneration 2020 15:60 -
Glycosphingolipids and neuroinflammation in Parkinson’s disease
Parkinson's disease is a progressive neurodegenerative disease characterized by the loss of dopaminergic neurons of the nigrostriatal pathway and the formation of neuronal inclusions known as Lewy bodies. Chro...
Citation: Molecular Neurodegeneration 2020 15:59 -
Neurofilaments in motor neuron disorders: towards promising diagnostic and prognostic biomarkers
Motor neuron diseases (MNDs) are etiologically and biologically heterogeneous diseases. The pathobiology of motor neuron degeneration is still largely unknown, and no effective therapy is available. Heterogene...
Citation: Molecular Neurodegeneration 2020 15:58 -
Higher CSF sTREM2 attenuates ApoE4-related risk for cognitive decline and neurodegeneration
The Apolipoprotein E ε4 allele (i.e. ApoE4) is the strongest genetic risk factor for sporadic Alzheimer’s disease (AD). TREM2 (i.e. Triggering receptor expressed on myeloid cells 2) is a microglial transmembra...
Citation: Molecular Neurodegeneration 2020 15:57 -
Integrated analysis of the aging brain transcriptome and proteome in tauopathy
Tau neurofibrillary tangle pathology characterizes Alzheimer’s disease and other neurodegenerative tauopathies. Brain gene expression profiles can reveal mechanisms; however, few studies have systematically ex...
Citation: Molecular Neurodegeneration 2020 15:56 -
Neuroimaging advances regarding subjective cognitive decline in preclinical Alzheimer’s disease
Subjective cognitive decline (SCD) is regarded as the first clinical manifestation in the Alzheimer’s disease (AD) continuum. Investigating populations with SCD is important for understanding the early patholo...
Citation: Molecular Neurodegeneration 2020 15:55 -
Correction to: Deciphering cellular transcriptional alterations in Alzheimer’s disease brains
An amendment to this paper has been published and can be accessed via the original article.
Citation: Molecular Neurodegeneration 2020 15:54 -
Behavioral and neural network abnormalities in human APP transgenic mice resemble those of App knock-in mice and are modulated by familial Alzheimer’s disease mutations but not by inhibition of BACE1
Alzheimer’s disease (AD) is the most frequent and costly neurodegenerative disorder. Although diverse lines of evidence suggest that the amyloid precursor protein (APP) is involved in its causation, the precis...
Citation: Molecular Neurodegeneration 2020 15:53 -
The Alzheimer’s disease-associated protective Plcγ2-P522R variant promotes immune functions
Microglia-specific genetic variants are enriched in several neurodegenerative diseases, including Alzheimer’s disease (AD), implicating a central role for alterations of the innate immune system in the disease...
Citation: Molecular Neurodegeneration 2020 15:52 -
Impaired NHEJ repair in amyotrophic lateral sclerosis is associated with TDP-43 mutations
Pathological forms of TAR DNA-binding protein 43 (TDP-43) are present in motor neurons of almost all amyotrophic lateral sclerosis (ALS) patients, and mutations in TDP-43 are also present in ALS. Loss and gain...
Citation: Molecular Neurodegeneration 2020 15:51 -
Peroxiredoxin 6 mediates protective function of astrocytes in Aβ proteostasis
Disruption of β-amyloid (Aβ) homeostasis is the initial culprit in Alzheimer’s disease (AD) pathogenesis. Astrocytes respond to emerging Aβ plaques by altering their phenotype and function, yet molecular mecha...
Citation: Molecular Neurodegeneration 2020 15:50 -
Two C-terminal sequence variations determine differential neurotoxicity between human and mouse α-synuclein
α-Synuclein (aSyn) aggregation is thought to play a central role in neurodegenerative disorders termed synucleinopathies, including Parkinson’s disease (PD). Mouse aSyn contains a threonine residue at position...
Citation: Molecular Neurodegeneration 2020 15:49 -
CNS axonal degeneration and transport deficits at the optic nerve head precede structural and functional loss of retinal ganglion cells in a mouse model of glaucoma
Glaucoma is a leading neurodegenerative disease affecting over 70 million individuals worldwide. Early pathological events of axonal degeneration and retinopathy in response to elevated intraocular pressure (I...
Citation: Molecular Neurodegeneration 2020 15:48 -
P2RX7 inhibitor suppresses exosome secretion and disease phenotype in P301S tau transgenic mice
Neuronal accumulation of misfolded microtubule-associated protein tau is a hallmark of neuropathology in Alzheimer’s disease, frontotemporal dementia, and other tauopathies, and has been a therapeutic target. ...
Citation: Molecular Neurodegeneration 2020 15:47 -
Cerebrospinal fluid profile of NPTX2 supports role of Alzheimer’s disease-related inhibitory circuit dysfunction in adults with Down syndrome
Alzheimer’s disease (AD) is the major cause of death in adults with Down syndrome (DS). There is an urgent need for objective markers of AD in the DS population to improve early diagnosis and monitor disease p...
Citation: Molecular Neurodegeneration 2020 15:46 -
The role of TDP-43 mislocalization in amyotrophic lateral sclerosis
Since its discovery as a primary component in cytoplasmic aggregates in post-mortem tissue of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP-43) has remained a central f...
Citation: Molecular Neurodegeneration 2020 15:45 -
BIN1 protein isoforms are differentially expressed in astrocytes, neurons, and microglia: neuronal and astrocyte BIN1 are implicated in tau pathology
Identified as an Alzheimer’s disease (AD) susceptibility gene by genome wide-association studies, BIN1 has 10 isoforms that are expressed in the Central Nervous System (CNS). The distribution of these isoforms in...
Citation: Molecular Neurodegeneration 2020 15:44 -
Integrated analysis of ultra-deep proteomes in cortex, cerebrospinal fluid and serum reveals a mitochondrial signature in Alzheimer’s disease
Based on amyloid cascade and tau hypotheses, protein biomarkers of different Aβ and tau species in cerebrospinal fluid (CSF) and blood/plasma/serum have been examined to correlate with brain pathology. Recentl...
Citation: Molecular Neurodegeneration 2020 15:43
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- ISSN: 1750-1326 (electronic)