Search Results - "prion"

Pathogenic mechanisms in prion disease

Table of Contents: “…Contents: Neurodegenerative diseases and protein misfolding disorders -- Prion disease in mice to study mechanisms of neurodegeneration due to misfolded protein accumulation -- Early prion neurodegenerative change is reversible: access to key neurotoxic pathways -- Critical change is loss of synaptic proteins due to activation of PERK branch of the unfolded protein response -- Modifying this pathway genetically is neuroprotective -- Modifying this pathway pharmacologically is neuroprotective -- Relevance of this pathway in other neurodegenerative diseases with misolded proteins e.g. …”
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Endoplasmic reticulum stress in neurodegenerative diseases

Table of Contents: “…Contents: Neurodegenerative disease -- Misfolded aggregates deposited in the brain -- Research strategy -- Prion diseases -- Two conformations of the prion protein -- Experimental scrapie model in mice -- Upregulation of ER chaperones -- ER-stress and UPR in human CJD -- PrP interaction with ER chaperones in brain -- PrPSc induction of ER-stress and neuronal death -- Calcineurin properties -- A novel treatment for prion diseases.…”
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Novel approaches to treating symptoms and slowing the progress of neurodegenerative diseases

Table of Contents: “…Contents: Brief overview of dementia -- Current treatments for Alzheimer's disease -- Role of muscarinic receptors in learning and memory -- Using prion disease as a model -- Effects of muscarinic receptor drugs on learning and memory deficits in prion disease -- Types of drugs for different stages of Alzheimer's disease.…”
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Expanding roles of RNA-binding proteins in neurodegenerative diseases

Table of Contents: “…Contents: Amyotrophic lateral sclerosis (ALS) -- Genetic causes of ALS -- Role of RNA-binding proteins and stress granules in ALS pathogenesis -- Emerging class of RNA-binding proteins with prion-like domains in neurodegenerative disease -- Mechanisms by which C9ORF72 mutations cause ALS.…”
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Neurodegenerative disease expression levels of normal sequence pathogenic proteins contribute to risk of disease /

Table of Contents: “…Contents: Protein pathology of disease -- Loci underlying autosomal dominant neurodegenerative disease -- Prion mutations causing Gerstmann Straussler syndrome and hereditary Creuzfeldt-Jakob disease -- Tau mutations cause autosomal dominant frontal temporal dementia -- Alpha-synuclein mutations cause autosomal dominant Parkinson's disease -- How genetic variability of these genes contributes to the risk of sporadic disease.…”
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Roles for Hsp40 molecular chaperones in protein misfolding disease

Table of Contents: “…Contents: Protein aggregation in conformational disease -- The Hsp40 system -- The Hsp70 system -- Small oligomers, prion, yeast, Huntington's disease and protein triage -- Mechanisms for proteotoxicity -- Mechanisms for amyloid disease.…”
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Clinical neuropathology text and color atlas / by Haberland, Catherine

Table of Contents: “…Basics of neuropathology -- Cerebral hypoxia -- Cerebrovascular diseases -- Neurodegenerative diseases -- Infectious diseases -- Prion diseases -- Demyelinating diseases -- Hereditary neurometabolic diseases -- Acquired neurometabolic diseases -- Tumors of the central nervous system -- Traumatic injuries of the central nervous system -- Congenital malformations of the central nervous system -- Fetal and perinatal cerebral pathology.…”
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Metal-based neurodegeneration : from molecular mechanisms to therapeutic strategies / by Crichton, Robert R.

Table of Contents: “…Brain function, physiology, and the blood-brain barrier -- Role of metal ions in brain function, metal transport, storage, and homeostasis -- Immune system and neuroinflammation -- Oxidative stress in neurodegenerative diseases -- Aging and mild cognitive impairment, MCI -- Parkinson's disease -- Alzheimer's disease -- Huntington's disease and polyglutamine expansion neurodegenerative diseases -- Friedreich's ataxia and diseases associated with expansion of non-coding triplets -- Creutzfeldt-Jakob and other prion diseases -- Amyotropic lateral sclerosis -- Alcoholic brain damage -- Other neurological diseases -- Therapeutic strategies to combat the onset and progression of neurological diseases.…”
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Protein degradation and defense against neurodegenerative disease part 2 of 2 /

Table of Contents: “…Contents: The 26S proteasome -- The 20S proteasome -- Proteasome inhibitors -- PS-341 -- Proteasome inhibitors in multiple myeloma -- Structures of the archaeal PAN ATPase-20S complex and the eukaryotic 26S proteasome -- Multiple ATP-dependent steps in proteasome function -- The gating mechanism of the proteasome -- Prions inhibit proteasome function -- Binding of ubiquitin conjugates activate the 26S proteasome by stimulating 20S gate-opening and ATP hydrolysis -- Ubiquitin conjugates activate their own degradation by the 26S Proteasome -- The inhibitor of proteasome-associated deubiquitinating enzyme Usp14, IU1, enhances degradation of tau.…”
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Targeting Aß oligomers a molecular basis for the cause, diagnosis, and treatment of Alzheimer's disease /

Table of Contents: “…Contents: The oligomer cascade hypothesis for Alzheimer's Disease -- Why the oligomer hypothesis has largely supplanted the amyloid cascade hypothesis -- The role of AβOs in memory failure and the major facets of AD neuropathology -- Mechanisms by which AβOs instigate neurotoxicity -- Why AβOs accumulate in the first place -- How AβOs offer superb targets for novel AD diagnostics and disease-modifying therapeutics -- Beside its direct link to Alzheimer's disease, the discovery of toxic Aβ oligomers has provided a novel structural archetype for toxins germane to more than two dozen diseases of protein mis-folding, including Diabetes, Parkinson's, and prion diseases.…”
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Merritt's neurology.

Table of Contents: “…Signs and symptoms in neurologic diagnosis: approach to the patient -- Delirium and confusion -- Memory loss, behavioral changes and dementia -- Aphasia, apraxia, and agnosia -- Syncope, seizures and their mimics -- Coma -- Headache -- Diagnosis of pain and paresthesias -- Dizziness, vertigo, and hearing loss -- Impaired vision -- Involuntary movements -- Syndromes caused by weak muscles -- Gait disorders -- CT and MRI -- Electroencephalography and evoked potentials -- Electromyography, nerve conduction studies, and and magnetic stimulation -- Autonomic testing -- Neurovascular imaging -- Endovascular neuroradiology -- Lumbar puncture and cerebrospinal fluid examination -- Muscle and nerve biopsy -- Neuropsychological evaluation -- DNA diagnosis -- Bacterial infections -- Focal infections -- Viral infections and postviral syndromes -- Human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS) -- Fungal infections -- Neurosarcoidosis -- Neurosyphilis -- Leptospirosis -- Lyme disease -- Parasitic infections -- Bacterial toxins -- Prion diseases -- Whipple disease -- Pathogenesis, classification, and epidemiology of cerebrovascular disease -- Examination of the patient with cerebrovascular disease -- Transient ischemic attack -- Cerebral infarction -- Intracerebral hemorrhage -- Genetics of stroke -- Other cerebrovascular syndromes -- Differential diagnosis of stroke -- Stroke in children -- Treatment and prevention of stroke -- Subarachnoid hemorrhage -- Cerebral venous and sinus thrombosis -- Vascular disease of the spinal cord -- Vasculitis -- Susac syndrome -- Vascular tumors and malformations -- Hydrocephalus -- Normal pressure hydrocephalus (NPH) -- Brain edema and disorders of intracranial pressure -- Superficial siderosis and intracerebral hypotension -- Hyperosmolar syndromes -- General considerations -- Tumors of the skull and cranial nerves -- Tumors of the meninges -- Gliomas -- Lymphomas -- Pineal region tumors -- Tumors of the pituitary gland -- Congenital and childhood tumors -- Metastatic tumors -- Spinal tumors -- Paraneoplastic syndromes -- Complications of cancer chemotherapy --…”
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