Not exact matches
The researchers, including first author Matheus Victor, PhD, a postdoctoral research associate, set out to generate
neurons that would mimic those
of adult patients in order to
model the onset and progression
of Huntington's
disease.
The research is also the first to demonstrate beneficial effects
of UDCA on dopaminergic
neurons, the nerve cells affected in Parkinson's
disease, in a fly
model of Parkinson's
disease which carries the same genetic change as some patients with the condition.
«Novel radiotracer measures olfactory
neurons in animal
models: Tracking deterioration in sense
of smell could provide early diagnosis
of neurodegenerative
disease.»
Experiments in mouse
models of Parkinson's
disease and amyotrophic lateral sclerosis (ALS, or Lou Gehrig's
disease) have demonstrated that Nrf2 - based neural protection is primarily conferred by astrocytes, but how
neurons might alert astrocytes to stressful conditions is poorly understood.
The study, published Feb. 16 in PLOS Biology, made use
of a mutant zebrafish strain that
models human Hirschsprung
disease, which is caused by loss
of the gut
neurons that coordinate gut contractions.
Several laboratories, including one led by Stewart Anderson
of the University
of Pennsylvania Perelman School
of Medicine, have demonstrated that transplanting inhibitory
neurons from healthy mice has improved symptoms in mice with
models of those
diseases.
This Purkinje
neuron was derived from patients with tuberous sclerosis and
model properties
of the
disease at the cellular and molecular level.
Our conversion process should
model late - onset aspects
of the
disease using
neurons derived from patients with the condition.»
Congratulations to Umrao Monani, Assistant Professor at the Motor
Neuron Center
of Columbia University and Cathleen Lutz, Associate Director
of Genetic Research Science at The Jackson Laboratory, and their colleagues for their new publication «Postsymptomatic Restoration
of SMN Rescues the
Disease Phenotype in a Mouse
Model of Severe Spinal Muscular Atrophy» in the Journal
of -LSB-...]
Here, Anna presents how studies
of neural stem cells and
neurons derived from iPS cells
of patients show faithful mimicking
of known
disease phenotypes in our cellular
models of disease, like Alzheimer's
disease, autism, and Down syndrome.
The paper «Nav1.1 - Overexpressing Interneuron Transplants Restore Brain Rhythms and Cognition in a Mouse
Model of Alzheimer's
Disease» was published online by
Neuron on March 15, 2018.
In 2002, the lab proved that transplanting embryonic stem cells in animal
models of Parkinson's
disease yielded functional new dopamine
neurons that restored motor function.
Dopamine
neurons derived from human ES cells efficiently engraft in animal
models of Parkinson's
disease
Loss
of mitochondrial complex I activity potentiates dopamine
neuron death induced by microtubule dysfunction in a Parkinson's
disease model.
The experiments, performed in rat
models of Parkinson's
disease, reveal that the latest version
of stem cell - derived dopamine cells fully mimic the characteristics and function
of the dopamine
neurons that are lost in Parkinson's
disease.
Most
of the study's experiments were done in the roundworm C. elegans, which have an SMN gene and motor
neurons — those that connect to muscle — that are very similar to humans, making them valuable
models in which to study this
disease.
In the study, which was conducted in collaboration with researchers at UC San Francisco and published today in the Journal
of Neuroscience, scientists transplanted inhibitory
neuron progenitors — early - stage brain cells that have the capacity to develop into mature inhibitory
neurons — into two mouse
models of Alzheimer's
disease, apoE4 or apoE4 with accumulation
of amyloid beta, another major contributor to Alzheimer's.
Because neurodegenerative disorders like PD are largely
diseases of aging,
modeling them in a culture dish using
neurons grown from iPS cells has been thought to be exceedingly difficult, if not impossible.
In the current study, the scientists discovered that, in a mouse
model of Alzheimer's
disease, the accumulation
of tau in
neurons disrupts the cells» ability to strengthen their connections with other
neurons, preventing them from stabilizing new memories.
Dr. Pfaff has developed a novel embryonic - stem cell - based
model of SMA that phenocopies the pathology
of human SMA, and can be used to study the basis
of the
disease and screen for compounds that might increase survival
of motor
neurons.
The review covers approaches such as spurring the production
of new
neurons and transplanting new
neurons while taking a look at the
disease -
modeling approaches and techniques that science is now using to refine approaches...
The findings demonstrate similarities in progressive muscle weakness and a decline in motor coordination in both
models of motor
neuron disease with a later onset and slower progression noted with PFN1 animals.
Using a
model of Leigh's
disease, a genetically inherited neurodegenerative disorder that affects mitochondria, the researchers tested energy levels in
neurons using the new assays.
Working with Ye, postdoctoral fellow Sung - Wuk Jang identified a compound called 7,8 - dihydroxyflavone that can duplicate BDNFâ $ ™ s effects on
neurons and can protect them against damage in animal
models of seizure, stroke and Parkinsonâ $ ™ s
disease.
Using mouse
models of MS, Dr. Akassoglou has shown that higher levels
of these proteins are early indicators
of MS and key contributors to
neuron damage, resulting in
disease progression.
These are
neurons in the eye that respond to decrements in light that we discovered in an animal
model are especially vulnerable early stages
of the
disease.
MIT's Li - Huei Tsai, Ed Boyden, and their colleagues have shown that stimulating
neurons to produce gamma waves at a frequency
of 40 Hz reduces the occurrence and severity
of several Alzheimer's - associated symptoms in a mouse
model of the
disease.
In a study published in
Neuron, Palop and his collaborators uncovered the therapeutic benefits
of genetically improving these interneurons and transplanting them into the brain
of a mouse
model of Alzheimer's
disease.
Differentiation from human pluripotent stem cells
of cortical
neurons of the superficial layers amenable to psychiatric
disease modeling and high - throughput drug screening.
Jorge Palop and his collaborators discovered that transplanting a special type
of neuron into the brain restores cognitive functions in mouse
models of Alzheimer's
disease.
So for our
model of this
disease, which we are currently developing, we have to generate a variety
of other brain cell types besides
neurons that are thought to foster immune responses in the brain.
Neurona's founders have discovered that certain types
of neuron transplants can rebalance nervous system activity, repair neural circuits, and rescue multiple animal
models of disease.
Duke s research will use patented multielectrode technology to directly measure the activity
of hundreds
of neurons to study brain dynamics in unique preclinical
models of diseases such as Parkinson s and schizophrenia.
For three years now we have been working on human cell
models of rare neurodegenerative
diseases with special emphasis on neuroacanthocytosis, neuronal ceroid lipofuscinosis as well as motor
neuron degeneration (using iPS cells).
DR can protect
neurons against degeneration in animal
models of Alzheimer's, Parkinson's and Huntington's
diseases and stroke.
Multiple studies show that the catechin compounds found in green tea can have various protecting effects on
neurons in test tubes and animals
models, potentially lowering the risk
of disease like Alzheimer's and Parkinson's.
No desirable effect was observed in an animal
model of amyotrophic lateral sclerosis (ALS), indicating that intermittent fasting has no beneficial effect on the development
of this motor
neuron disease (51).
Some phytochemicals which induce the Keap1 / Nrf2 pathway and have demonstrated protective effects in both
neuron tissue culture and animal
models of Parkinson's and neurodegenerative
disease include:
For example, KBs were recently reported to act as neuroprotective agents by raising ATP levels and reducing the production
of reactive oxygen species in neurological tissues, 80 together with increased mitochondrial biogenesis, which may help to enhance the regulation
of synaptic function.80 Moreover, the increased synthesis
of polyunsaturated fatty acids stimulated by a KD may have a role in the regulation
of neuronal membrane excitability: it has been demonstrated, for example, that polyunsaturated fatty acids modulate the excitability
of neurons by blocking voltage-gated sodium channels.81 Another possibility is that by reducing glucose metabolism, ketogenic diets may activate anticonvulsant mechanisms, as has been reported in a rat
model.82 In addition, caloric restriction per se has been suggested to exert neuroprotective effects, including improved mitochondrial function, decreased oxidative stress and apoptosis, and inhibition
of proinflammatory mediators, such as the cytokines tumour necrosis factor - α and interleukins.83 Although promising data have been collected (see below), at the present time the real clinical benefits
of ketogenic diets in most neurological
diseases remain largely speculative and uncertain, with the significant exception
of its use in the treatment
of convulsion
diseases.
D - beta - hydroxybutyrate protects
neurons in
models of Alzheimer's and Parkinson's
disease.