Birgit Hutter‐Paier
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View article: Montelukast alleviates neuroinflammation and improves motor functions in the line 61 model of Parkinson's disease: An exploratory study
Montelukast alleviates neuroinflammation and improves motor functions in the line 61 model of Parkinson's disease: An exploratory study Open
Parkinson's disease (PD) is a neurodegenerative movement disorder of high global burden. Uncertainties regarding its exact etiology have been hindering the development of curative therapies. As microglia, the brain's immune cells, are susp…
View article: Cerebral Amyloid Angiopathy like pathology accompanied by intense gliosis in T2D‐induced APPxhQC mice
Cerebral Amyloid Angiopathy like pathology accompanied by intense gliosis in T2D‐induced APPxhQC mice Open
Background Cerebral Amyloid Angiopathy (CAA), characterized by the presence of amyloid β (Aβ) deposits in cerebral blood vessels has been associated with cognitive impairment and Alzheimer’s disease (AD). Vascular risk factors, such as typ…
View article: Sex and genotype dependent differences in amyloid beta levels and lipid metabolism in type 2 diabetic APPxhQC transgenic mice
Sex and genotype dependent differences in amyloid beta levels and lipid metabolism in type 2 diabetic APPxhQC transgenic mice Open
Background Accumulation of amyloid beta (Aβ) and tau proteins have for decades been thought to be central in the pathogenesis of Alzheimer’s disease (AD). More recently, a plethora of evidence emerged that links metabolic dysfunctions such…
View article: Astaxanthin enhances autophagy, amyloid beta clearance and exerts anti-inflammatory effects in in vitro models of Alzheimer’s disease-related blood brain barrier dysfunction and inflammation
Astaxanthin enhances autophagy, amyloid beta clearance and exerts anti-inflammatory effects in in vitro models of Alzheimer’s disease-related blood brain barrier dysfunction and inflammation Open
Defective degradation and clearance of amyloid-β as well as inflammation per se are crucial players in the pathology of Alzheimer's disease (AD). A defective transport across the blood-brain barrier is causative for amyloid-β (Aβ) accumula…
View article: Evaluating the effect of R-Baclofen and LP-211 on autistic behavior of the BTBR and Fmr1-KO mouse models
Evaluating the effect of R-Baclofen and LP-211 on autistic behavior of the BTBR and Fmr1-KO mouse models Open
Introduction Autism spectrum disorder (ASD) is a persistent neurodevelopmental condition characterized by two core behavioral symptoms: impaired social communication and interaction, as well as stereotypic, repetitive behavior. No distinct…
View article: Editorial: Neuronopathic lysosomal storage diseases - specific neuronal characteristics and therapeutic approaches
Editorial: Neuronopathic lysosomal storage diseases - specific neuronal characteristics and therapeutic approaches Open
EDITORIAL article Front. Mol. Neurosci., 08 November 2022Sec. Brain Disease Mechanisms https://doi.org/10.3389/fnmol.2022.1078804
View article: Leukotriene Signaling as a Target in α-Synucleinopathies
Leukotriene Signaling as a Target in α-Synucleinopathies Open
Parkinson’s disease (PD) and dementia with Lewy bodies (DLB) are two common types of α-synucleinopathies and represent a high unmet medical need. Despite diverging clinical manifestations, both neurodegenerative diseases share several face…
View article: Impaired Retromer Function in Niemann-Pick Type C Disease Is Dependent on Intracellular Cholesterol Accumulation
Impaired Retromer Function in Niemann-Pick Type C Disease Is Dependent on Intracellular Cholesterol Accumulation Open
Niemann-Pick type C disease (NPC) is a rare inherited neurodegenerative disorder characterized by an accumulation of intracellular cholesterol within late endosomes and lysosomes due to NPC1 or NPC2 dysfunction. In this work, we tested the…
View article: Combination of the Glutaminyl Cyclase Inhibitor PQ912 (Varoglutamstat) and the Murine Monoclonal Antibody PBD-C06 (m6) Shows Additive Effects on Brain Aβ Pathology in Transgenic Mice
Combination of the Glutaminyl Cyclase Inhibitor PQ912 (Varoglutamstat) and the Murine Monoclonal Antibody PBD-C06 (m6) Shows Additive Effects on Brain Aβ Pathology in Transgenic Mice Open
Compelling evidence suggests that pyroglutamate-modified Aβ (pGlu3-Aβ; AβN3pG) peptides play a pivotal role in the development and progression of Alzheimer’s disease (AD). Approaches targeting pGlu3-Aβ by glutaminyl cyclase (QC) inhibition…
View article: Characterization of an APP/tau rat model of Alzheimer’s disease by positron emission tomography and immunofluorescent labeling
Characterization of an APP/tau rat model of Alzheimer’s disease by positron emission tomography and immunofluorescent labeling Open
Background To better understand the etiology and pathomechanisms of Alzheimer’s disease, several transgenic animal models that overexpress human tau or human amyloid-beta (Aβ) have been developed. In the present study, we generated a novel…
View article: Evaluation of Neuropathological Features in the SOD1-G93A Low Copy Number Transgenic Mouse Model of Amyotrophic Lateral Sclerosis
Evaluation of Neuropathological Features in the SOD1-G93A Low Copy Number Transgenic Mouse Model of Amyotrophic Lateral Sclerosis Open
Amyotrophic lateral sclerosis (ALS) still depicts an incurable and devastating disease. Drug development efforts are mostly based on superoxide dismutase 1 gene (SOD1)-G93A mice that present a very strong and early phenotype, allowing only…
View article: The Leukotriene Receptor Antagonist Montelukast Attenuates Neuroinflammation and Affects Cognition in Transgenic 5xFAD Mice
The Leukotriene Receptor Antagonist Montelukast Attenuates Neuroinflammation and Affects Cognition in Transgenic 5xFAD Mice Open
Alzheimer’s disease (AD) is the most common form of dementia. In particular, neuroinflammation, mediated by microglia cells but also through CD8+ T-cells, actively contributes to disease pathology. Leukotrienes are involved in neuroinflamm…
View article: Homozygosity of BACHD rats not only causes strong behavioral deficits in young female rats but also a reduced breeding success
Homozygosity of BACHD rats not only causes strong behavioral deficits in young female rats but also a reduced breeding success Open
Huntington's disease is known to be a purely genetic disease based on an expansion of a CAG base triplet repeat in the coding region of the Huntingtin gene. Some years ago, researchers were able to introduce the extensive full-length gene …
View article: Metabolic, Phenotypic, and Neuropathological Characterization of the Tg4-42 Mouse Model for Alzheimer’s Disease
Metabolic, Phenotypic, and Neuropathological Characterization of the Tg4-42 Mouse Model for Alzheimer’s Disease Open
Background: Preclinical Alzheimer’s disease (AD) research strongly depends on transgenic mouse models that display major symptoms of the disease. Although several AD mouse models have been developed representing relevant pathologies, only …
View article: Quantification of Huntington’s Disease Related Markers in the R6/2 Mouse Model
Quantification of Huntington’s Disease Related Markers in the R6/2 Mouse Model Open
Huntington’s disease (HD) is caused by an expansion of CAG triplets in the huntingtin gene, leading to severe neuropathological changes that result in a devasting and lethal phenotype. Neurodegeneration in HD begins in the striatum and spr…
View article: Improved Bioavailability of Montelukast through a Novel Oral Mucoadhesive Film in Humans and Mice
Improved Bioavailability of Montelukast through a Novel Oral Mucoadhesive Film in Humans and Mice Open
The leukotriene receptor antagonist Montelukast (MTK) is an approved medication for the treatment of asthma and allergic rhinitis. The existing marketed tablet forms of MTK exhibit inconsistent uptake and bioavailability, which partially e…
View article: Correlation of Aβ‐pE(3) and ptau in human and mouse brain
Correlation of Aβ‐pE(3) and ptau in human and mouse brain Open
Background Senile plaques frequently contain pyroglutamate amyloid β (Aβ‐pE(3)), a N‐terminally truncated Aβ species that is more closely linked to Alzheimer’s Disease (AD) compared to other Aβ species. Tau protein is highly phosphorylated…
View article: Neurofilament‐light chain in murine models of neurodegenerative and rare diseases
Neurofilament‐light chain in murine models of neurodegenerative and rare diseases Open
Background Neurofilament‐light chain (NF‐L) is known as biomarker of many neurodegenerative diseases and their progression. By analyzing amyotrophic lateral sclerosis (ALS) patients CSF or plasma progression of NF‐L levels can forecast con…
View article: Improved bioavailability of montelukast through a novel oral mucoadhesive film in humans and mice
Improved bioavailability of montelukast through a novel oral mucoadhesive film in humans and mice Open
The leukotriene receptor antagonist Montelukast (MTK) is an approved medication for the treatment of asthma and allergic rhinitis. The existing marketed tablet forms of MTK exhibit inconsistent uptake and bioavailability, which partially e…
View article: Constant Levels of Tau Phosphorylation in the Brain of htau Mice
Constant Levels of Tau Phosphorylation in the Brain of htau Mice Open
Excessive tau phosphorylation is the hallmark of tauopathies. Today's research thus focusses on the development of drugs targeting this pathological feature. To test new drugs in preclinical studies, animal models are needed that properly …
View article: Correlation of pyroglutamate amyloid β and ptau Ser202/Thr205 levels in Alzheimer’s disease and related murine models
Correlation of pyroglutamate amyloid β and ptau Ser202/Thr205 levels in Alzheimer’s disease and related murine models Open
Senile plaques frequently contain Aβ-pE(3), a N-terminally truncated Aβ species that is more closely linked to AD compared to other Aβ species. Tau protein is highly phosphorylated at several residues in AD, and specifically phosphorylatio…
View article: Neurofilament-Light Chain as Biomarker of Neurodegenerative and Rare Diseases With High Translational Value
Neurofilament-Light Chain as Biomarker of Neurodegenerative and Rare Diseases With High Translational Value Open
Neurofilament-light chain (NF-L) is a well-known clinical biomarker of many neurodegenerative diseases. By analyzing amyotrophic lateral sclerosis (ALS) patients cerebrospinal fluid (CSF) or plasma, progression of NF-L levels can forecast …
View article: CD8+ T-cells infiltrate Alzheimer’s disease brains and regulate neuronal- and synapse-related gene expression in APP-PS1 transgenic mice
CD8+ T-cells infiltrate Alzheimer’s disease brains and regulate neuronal- and synapse-related gene expression in APP-PS1 transgenic mice Open
Neuroinflammation is a major contributor to disease progression in Alzheimer's disease (AD) and is characterized by the activity of brain resident glial cells, in particular microglia cells. However, there is increasing evidence that perip…
View article: Transgene integration causes RARB downregulation in homozygous Tg4–42 mice
Transgene integration causes RARB downregulation in homozygous Tg4–42 mice Open
Alzheimer’s disease can be modelled by different transgenic mouse strains. To gain deeper insight into disease model mechanisms, the previously described Tg4–42 mouse was analysed for transgene integration. On RNA/DNA level the transgene i…
View article: Plasma pharmacokinetic and metabolism of [18F]THK-5317 are dependent on sex
Plasma pharmacokinetic and metabolism of [18F]THK-5317 are dependent on sex Open
Sex-specific differences might also be present in humans and thus clinical trials should have adequate sample size to account for effects in men and women separately.
View article: Characterization of the visceral and neuronal phenotype of 4L/PS-NA mice modeling Gaucher disease
Characterization of the visceral and neuronal phenotype of 4L/PS-NA mice modeling Gaucher disease Open
Gaucher disease is caused by a deficiency in glucocerebrosidase that can result in non-neuronal as well as neuronal symptoms. Common visceral symptoms are an increased organ size, specifically of the spleen, and glucosylceramide as well as…