Here researchers were able to work with
a human cellular model genetically identical to that of the patients.
Interestingly, when compared to
human cellular models, mouse models of PD did not demonstrate the same toxic cascade, according to the study.
Monogenic diseases are often lacking of physiological
human cellular models dedicated to the study of the molecular mechanism implicated in the development of the pathology.
«We haven't had
human cellular models that faithfully reproduce this feature of the disease,» said Gottesfeld.
The project will analyse the chemical changes that occur in Unkempt in
human cellular models of TSC.
Not exact matches
The disruption of prenatal
cellular activity in zebra fish, which share 80 percent of their genes with
humans and are considered a good
model for studying
human brain development, seemed to result in hyperactivity, according to the Canadian study, which was published Monday in the Proceedings of the National Academy of Sciences.
The scientists are testing the ability of drugs already on the market to reverse this
cellular transformation in the pancreas in mice
models of
human pancreatic cancer.
Now, in this week's issue of the Proceedings of the National Academy of Sciences, Thomas Albright and Ricardo Gil - da - Costa of the Salk Institute for Biological Studies describe a
model system that completes the bridge between
cellular and
human studies of schizophrenia, an advance that should help speed the development of therapeutics for schizophrenia and other neurological disorders.
While scientists could do
cellular work in animal
models on the role of dopamine versus glutamate, and they could do EEGs in
human beings, a bridge between the two remained elusive.
Currently, Deng's laboratory is conducting additional preclinical studies using the
human - derived stem cells from Down syndrome patients and mouse
models to determine whether
cellular and behavioral abnormalities can be improved with minocycline therapy and other candidate drugs.
To do this, they created a
cellular model of Werner syndrome by using a cutting - edge gene - editing technology to delete WRN gene in
human stem cells.
For instance, CiRA's Kohei Yamamizu recently reported developing a
cellular model of the blood — brain barrier made entirely from
human iPS cells.
With CiPA,
human iPSC - cardiomyocytes are employed to assess drug effects on the cardiac action potential to confirm the outcome of in silico
modeling efforts, relying on hiPSC - cardiomyocytes to provide the integrated drug response from multiple native
human currents in a
human - derived
cellular preparation.
The researchers hope their new cell lines will be a useful resource for studying the
cellular and molecular intricacies of Huntington's further, and suggest they may provide a
model for examining other diseases of the brain that are specific to
humans.
Drosophila serves as a
model organism that helps geneticists to decode the molecular fundamentals of
cellular biology and unravel mechanisms that are conserved in
human beings and other vertebrates.
It's now possible to not only
model disease using the cells, but also to compare iPSCs from
humans to those of our closest living relatives --- great apes, with which we share a majority of genes --- for insight into what molecular and
cellular features make us
human.
Dr. Maria Iribarne, first author of this study, commented: «The gosh mutant is a good
model for understanding the molecular and
cellular mechanism of cone cell death and the pathological process of
human LCA.
«The development of this microfluidic lung
model, as well as other organs - on - chip, holds the promise of improving the physiological relevance of
cellular models for more accurate prediction of the effects of toxicants and drugs on
humans, and for reducing the use of animals in medical and pharmaceutical research,» said Sonia Grego, Ph.D., research scientist at RTI and the project's principal investigator.
Dr. Chartoff's Neurobiology of Motivated Behavior Laboratory, founded in 2009, investigates the molecular, biochemical, and
cellular mechanisms underlying drug dependence and withdrawal using animal behavioral
models with high relevance to the
human condition.
One challenge has been the lack of animal and
cellular models that can precisely replicate the
human disease for scientific investigation.
Human Embryonic Stem (hES) cells derived from Preimplantation Genetic Diagnosed (PGD)- embryos offer a new alternative source of
cellular model as they can be largely expanded, differentiated in several cell types and harbors «naturally» the causative mutation of the pathology.
Molecular mechanism underlying diseases (conformational diseases, cystic kidney disorders, neuromuscular diseases, inflammation, iron - related diseases, disorders of
human reproduction, osteoporosis and bone diseases) are being investigated by many groups with robust animal and
cellular models and patients» biological samples.
We investigate
cellular and molecular mechanisms of retinal degeneration and regeneration using rodents and
human cells as
model systems.
We address these fundamental biological questions at the organismal level, utilizing relevant
models of
human cancer, and at a
cellular and detailed molecular structural level.
To achieve this, I am developing novel
cellular and animal
models (like the one proposed here) that better reflect the new biological insights stemming from
human genetics.
We are now developing animal
models that recapitulate
human obesity mutations in an effort to determine the
cellular factors that underlie PC1 / 3 - mediated obesity.
Sergiu P. Pasca, 36, assistant professor of psychiatry and behavioral sciences at Stanford, uses
models of the
human brain — created through
cellular reprogramming technology — to explore the biological underpinnings of brain diseases like autism.
He has developed novel
cellular models of neurological diseases (schizophrenia, MS, autism, Alzheimer's, Parkinson's) using
human induced pluripotent stem cells (iPSCs) derived from donor patients and differentiated to specific neuronal cell types.
The Alliance brings together the efforts of the major National Institutes of Health (NIH) National
Human Genome Research Institute (NHGRI)- funded
Model Organism Database (MOD) groups, and the Gene Ontology (GO) Consortium, in a synergistic integration of expertly - curated information about the functioning of
cellular systems.
Telomere length predicts both
cellular health and disease in rodent
models and
humans.8 Shorter telomeres predict onset of cardiometabolic diseases of aging.9 Chronic stress is associated with higher inflammation, shorter telomeres, and lower activity levels of telomerase, the
cellular enzyme that elongates telomeric DNA.10, 11 Levels of amyloid beta (Aβ) proteins circulating in the blood appear to be stress - related in rodent
models12 and may be affected by stress reduction, and greater Aβ42 / Aβ40 ratios are associated with lower risk of dementia.13
She continued to develop these
models and to investigate the molecular and
cellular mechanisms underlying
human tumor dormancy as an instructor in Folkman's lab and later as an Assistant Professor at the Center of Cancer Systems Biology at Tufts University School of Medicine, Boston.
Dr. Apte notes that current research suggests that NAD + biosynthesis is a critical process in
cellular function and homeostasis in both animal
models and
humans.
Flavopiridol inhibits several
cellular kinases and has demonstrated cytostatic and cytotoxic activity in vitro and in vivo in numerous
human tumor cell lines and xenograft
models (including
human breast, prostate, and lung carcinoma) at clinically achievable concentrations.
Design and Methods Taking advantage of a
model of spontaneous
cellular transformation, we sequenced JAK1 in selected tumorigenic BaF3 clones and identified 25 de novo JAK1 activating mutations, including 5 mutations already described in
human leukemias.
The advent of
human - induced pluripotent stem cell (hiPSC) technology has provided a unique opportunity to establish
cellular models of disease from individual patients, and to study the effects of the underlying genetic aberrations upon multiple different cell types, many of which would not normally be accessible.
These findings have identified an alternative source of replacement tissue for use in
human retinal
cellular therapies, and provide a new in vitro
cellular model system in which to study RPE diseases affecting
human patients.
Technology utilizing
human induced pluripotent stem cells (iPS cells) has enormous potential to provide improved
cellular models of
human disease.
The authors of the study explain that their findings suggest that ketogenic diets could also potentially help normalize pathological behaviors in the
human model of schizophrenia by providing alternative energy sources via ketones, the products of fat breakdown, which would substitute the abnormally functioning
cellular energy pathways in the brains of people suffering from this disease.
Bottom line While research has shown some benefits with regards to ER + breast cancer cell death and prevention of metastases within mice and
cellular models, it is recommended that
human intake should be through diet only, not supplementation.