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UCLA researchers reveal unusual chemistry of protein with role in neurodegenerative disorders

A common feature of neurodegenerative diseases is the formation of permanent tangles of insoluble proteins in cells. The beta-amyloid plaques found in people with Alzheimer ’s disease and the inclusion bodies in motor neurons in the brains of people with amyotrophic lateral sclerosis are two examples. Those aggregates, and others like them, can kill cells and lead to debilitating and progressive neurodegenerative diseases.A study by Douglas Black and colleagues in UCLA ’s department of microbiology, immunology and molecular genetics, reveals that not all protein aggregates in brain cells are toxic. Their paper, published in the journal Cell, reports that an RNA-binding protein called Rbfox1, which is abundant in the brain, undergoes an unusual chemical transform ation to form nontoxic aggregates inside neurons, and that this aggregation is needed for Rbfox1 to perform its essential function, which is splicing RNA during the gene expression process.Mutations in the Rbfox1 gene are linked with some forms of familial epilepsy and autism spectrum disorder, so scientists are interested in understanding how Rbfox1 controls splicing in the brain. The new discovery is also important because the biochemistry of Rbfox1 is similar to those of proteins that are believed to play roles in several neurodegenerative disorders, including a protein called FUS, which aggregates in amyotrophic lateral sclerosis, also known as ALS or Lou Gehrig ’s disease.In people with ALS, FUS...
Source: UCLA Newsroom: Health Sciences - Category: Universities & Medical Training Source Type: news

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Authors: Reible B, Schmidmaier G, Prokscha M, Moghaddam A, Westhauser F Abstract Bone defect treatment belongs to the most challenging fields in orthopedic surgery and requires the well-coordinated application of mesenchymal stem cells (MSC) and differentiation factors. MSC isolated from reaming material (RMSC) and iliac crest (BMSC) in combination with bone morphogenetic protein-7 (BMP-7) and insulin-like growth factor-1 (IGF-1) have been used. The short half-life of both factors limit their applications: a burst release of the factor can probably not induce sustainable differentiation. We stimulated MSC in osteog...
Source: Growth Factors - Category: Research Tags: Growth Factors Source Type: research
n Maître As pluripotent stem cells become primed to give rise to all bodily cell types, they begin to form the amniotic cavity in which the mammalian fetus will grow. A checkpoint that gates this transition has now been identified.
Source: Nature - Category: Research Authors: Tags: News & Views Source Type: research
"What we have is the new standard of care for how students with autism can and should be served," the Bancroft CEO said.
Source: bizjournals.com Health Care News Headlines - Category: Health Management Authors: Source Type: news
Lengthy delays in epilepsy diagnosis are contributing to preventable motor vehicle accidents (MVAs), new research suggests.Medscape Medical News
Source: Medscape Medical News Headlines - Category: Consumer Health News Tags: Neurology & Neurosurgery News Source Type: news
Conditions:   Epilepsy;   Anxiety Intervention:   Drug: Clobazam Sponsor:   Hugo W. Moser Research Institute at Kennedy Krieger, Inc. Recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Conditions:   Hemorrhagic Stroke;   Intracerebral Hemorrhage Intervention:   Biological: MSC Sponsor:   Mayo Clinic Recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
The objective of this study will be to synthesize the epidemiological evidence and evaluate the validity of the associations between central nervous system disorders and the risk of developing or dying from cancer.Methods/designWe will perform an umbrella review of systematic reviews and conduct updated meta-analyses of observational studies (cohort and case-control) investigating the association between central nervous system disorders and the risk of developing or dying from any cancer or specific types of cancer. Searches involving PubMed/MEDLINE, EMBASE, SCOPUS and Web of Science will be used to identify systematic rev...
Source: Systematic Reviews - Category: Global & Universal Source Type: research
Abstract Matricellular proteins (MCPs) are actively expressed non-structural proteins present in the extracellular matrix, which rapidly turnover and possess regulatory roles, as well as mediate cell-cell interactions. MCPs characteristically contain binding sites for other extracellular proteins, cell surface receptors, growth factors, cytokines and proteases, that provide structural support for surrounding cells. MCPs are present in most organs, including brain, and play a major role in cell-cell interactions and tissue repair. Among the MCPs found in brain include thrombospondin-1/2, secreted protein acidic and...
Source: Neurochemical Research - Category: Neuroscience Authors: Tags: Neurochem Res Source Type: research
Abstract Kir4.1 is an inwardly rectifying K+ channel expressed exclusively in glial cells in the central nervous system. In glia, Kir4.1 is implicated in several functions including extracellular K+ homeostasis, maintenance of astrocyte resting membrane potential, cell volume regulation, and facilitation of glutamate uptake. Knockout of Kir4.1 in rodent models leads to severe neurological deficits, including ataxia, seizures, sensorineural deafness, and early postnatal death. Accumulating evidence indicates that Kir4.1 plays an integral role in the central nervous system, prompting many laboratories to study the p...
Source: Acta Neuropathologica - Category: Neurology Source Type: research
Epilepsy is a common disease that affects 1 in 26 individuals in their lifetime. According to a National Institute of Neurological Disorders and Stroke assessment, with 2 million affected individuals, epilepsy ranks only fourth to migraine, stroke, and Alzheimer disease in the prevalence of neurological disorders. Epilepsy affects more people than autism, amyotrophic lateral sclerosis, cerebral palsy, multiple sclerosis, and Parkinson disease combined. Approximately 0.3% to 0.5% of all pregnancies are among women with epilepsy (WWE). The risks during pregnancy in WWE have been uncertain.
Source: JAMA Neurology - Category: Neurology Source Type: research
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