scholarly journals Neuroinvasion in Prion Diseases: The Roles of Ascending Neural Infection and Blood Dissemination

2010 ◽  
Vol 2010 ◽  
pp. 1-16 ◽  
Author(s):  
Sílvia Sisó ◽  
Lorenzo González ◽  
Martin Jeffrey
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Prion Protein ◽  
Prion Diseases ◽  
Autonomic Nerves ◽  
Peripheral Organs ◽  
Prion Strains ◽  
New Variant ◽  
Jakob Disease ◽  
The Central Nervous System

Prion disorders are infectious, neurodegenerative diseases that affect humans and animals. Susceptibility to some prion diseases such as kuru or the new variant of Creutzfeldt-Jakob disease in humans and scrapie in sheep and goats is influenced by polymorphisms of the coding region of the prion protein gene, while other prion disorders such as fatal familial insomnia, familial Creutzfeldt-Jakob disease, or Gerstmann-Straussler-Scheinker disease in humans have an underlying inherited genetic basis. Several prion strains have been demonstrated experimentally in rodents and sheep. The progression and pathogenesis of disease is influenced by both genetic differences in the prion protein and prion strain. Some prion diseases only affect the central nervous system whereas others involve the peripheral organs prior to neuroinvasion. Many experiments undertaken in different species and using different prion strains have postulated common pathways of neuroinvasion. It is suggested that prions access the autonomic nerves innervating peripheral organs and tissues to finally reach the central nervous system. We review here published data supporting this view and additional data suggesting that neuroinvasion may concurrently or independently involve the blood vascular system.

scholarly journals Postmortem Quantitative Analysis of Prion Seeding Activity in the Digestive System

Molecules ◽  
2019 ◽  
Vol 24 (24) ◽  
pp. 4601 ◽  
Author(s):  
Katsuya Satoh ◽  
Takayuki Fuse ◽  
Toshiaki Nonaka ◽  
Trong Dong ◽  
Masaki Takao ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Quantitative Analysis ◽  
Prion Protein ◽  
Neurodegenerative Disorders ◽  
Digestive System ◽  
Prion Diseases ◽  
Jakob Disease ◽  
The Central Nervous System ◽  
Almost All

Human prion diseases are neurodegenerative disorders caused by prion protein. Although infectivity was historically detected only in the central nervous system and lymphoreticular tissues of patients with sporadic Creutzfeldt-Jakob disease, recent reports suggest that the seeding activity of Creutzfeldt-Jakob disease prions accumulates in various non-neuronal organs including the liver, kidney, and skin. Therefore, we reanalyzed autopsy samples collected from patients with sporadic and genetic human prion diseases and found that seeding activity exists in almost all digestive organs. Unexpectedly, activity in the esophagus reached a level of prion seeding activity close to that in the central nervous system in some CJD patients, indicating that the safety of endoscopic examinations should be reconsidered.

Human prion diseases with variant prion protein

1994 ◽  
Vol 343 (1306) ◽  
pp. 391-398 ◽  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Prion Protein ◽  
Prion Diseases ◽  
Molecular Genetic ◽  
Distinct Type ◽  
Plaque Type ◽  
The Central Nervous System ◽  
Human Prion Protein ◽  
Synaptic Structures

Recent molecular genetic studies revealed that the human prion protein (PrP) gene has a large repertoire of polymorphisms and mutations. Each variant PrP seems to correspond to a distinct type of prion diseases. W e report herein that it is useful to classify prion diseases into plaque type or non-plaque type, based on the distribution of PrP in the central nervous system. The variant PrP including codon 102, codon 105, codon 129, codon 145 and insertional polymorphisms belong to the plaque type prion diseases, whereas the wild-type PrP and the variants including codon 180, codon 200, and codon 232 polym orphisms belong to the non-plaque type. T he non-plaque type prion diseases showed a rapidly progressive dem entia, myoclonus and periodic synchronous discharges in the electroencephalogram , and in the pathological findings diffuse grey matter PrP accumulations including the synaptic structures. The plaque type prion diseases showed a long clinical course without myoclonus and periodic synchronous discharges, and the major PrP accumulation sites were extracellular PrP plaques. The distribution of PrP deposits in the central nervous system influences the clinical and pathological aspects of prion diseases. Thus, PrP accumulations may play a central role in the pathogenesis of prion diseases.

scholarly journals Risdiplam distributes and increases SMN protein in both the central nervous system and peripheral organs

2018 ◽  
Vol 6 (6) ◽  
pp. e00447 ◽  
Author(s):  
Agnès Poirier ◽  
Marla Weetall ◽  
Katja Heinig ◽  
Franz Bucheli ◽  
Kerstin Schoenlein ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Peripheral Organs ◽  
The Central Nervous System ◽  
Smn Protein

scholarly journals Aspergillosis of the central nervous system in a previously healthy patient that simulated Creutzfeldt-Jakob disease

2016 ◽  
Vol 7 (40) ◽  
pp. 940 ◽  
Author(s):  
SergioV Esparza-Gutiérrez ◽  
Adrián Santana-Ramírez ◽  
Pedro Avila-Rodríguez ◽  
JEugenio Jiménez-Gómez ◽  
Ezequiel Vélez-Gómez ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Healthy Patient ◽  
Jakob Disease ◽  
The Central Nervous System

Primary Angiitis of the Central Nervous System Mimicking Sporadic Creutzfeldt-Jakob Disease

2018 ◽  
Vol 32 (3) ◽  
pp. 258-261
Author(s):  
Dian He ◽  
Gang Cai ◽  
Yan Li ◽  
Qi Liu ◽  
Kang Xiao ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Primary Angiitis ◽  
Jakob Disease ◽  
The Central Nervous System

The neuroepidemiology of human prion disease

2020 ◽  
pp. 367-378
Author(s):  
Patrick JM Urwin ◽  
Anna M Molesworth
Keyword(s):  
Prion Protein ◽  
Prion Disease ◽  
Protein Misfolding ◽  
Prion Diseases ◽  
Prion Protein Gene ◽  
Human Prion Disease ◽  
Disease States ◽  
Jakob Disease ◽  
The Central Nervous System ◽  
Sporadic Disease

Human prion diseases comprise a number of rare and fatal neurodegenerative conditions that result from the accumulation in the central nervous system of an abnormal form of a naturally occurring protein, called the prion protein. The diseases occur in genetic, sporadic, and acquired forms: genetic disease is associated with mutations in the prion protein gene (PRNP); sporadic disease is thought to result from a spontaneous protein misfolding event; acquired disease results from transmission of infection from an animal or another human. The potential transmissibility of the prion in any of these forms, either in disease states or during the incubation period, has implications for public health. Here we focus on Creutzfeldt-Jakob Disease (CJD), including variant Creutzfeldt-Jakob Disease (vCJD), although we will also discuss other forms of human prion disease.

scholarly journals Impact of the Autonomic Nervous System on the Skeleton

2018 ◽  
Vol 98 (3) ◽  
pp. 1083-1112 ◽  
Author(s):  
Florent Elefteriou
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Bone Mass ◽  
Bone Remodeling ◽  
Cancer Metastasis ◽  
Bone Development ◽  
Autonomic Nerves ◽  
Bone Homeostasis ◽  
Bone Mass Accrual ◽  
The Central Nervous System

It is from the discovery of leptin and the central nervous system as a regulator of bone remodeling that the presence of autonomic nerves within the skeleton transitioned from a mere histological observation to the mechanism whereby neurons of the central nervous system communicate with cells of the bone microenvironment and regulate bone homeostasis. This shift in paradigm sparked new preclinical and clinical investigations aimed at defining the contribution of sympathetic, parasympathetic, and sensory nerves to the process of bone development, bone mass accrual, bone remodeling, and cancer metastasis. The aim of this article is to review the data that led to the current understanding of the interactions between the autonomic and skeletal systems and to present a critical appraisal of the literature, bringing forth a schema that can put into physiological and clinical context the main genetic and pharmacological observations pointing to the existence of an autonomic control of skeletal homeostasis. The different types of nerves found in the skeleton, their functional interactions with bone cells, their impact on bone development, bone mass accrual and remodeling, and the possible clinical or pathophysiological relevance of these findings are discussed.

The Neuroimmune Interface in Prion Diseases

Physiology ◽  
2000 ◽  
Vol 15 (5) ◽  
pp. 250-255
Author(s):  
Michael A. Klein ◽  
Adriano Aguzzi
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Immune System ◽  
Prion Disease ◽  
Neurodegenerative Disorders ◽  
Prion Diseases ◽  
The Central Nervous System

Prion diseases are fatal neurodegenerative disorders of animals and humans. Here we address the role of the immune system in the spread of prions from peripheral sites to the central nervous system and its potential relevance to iatrogenic prion disease.

EFFECT OF SEROTONIN AND NEUROPEPTIDES ON ADENYLATE CYCLASE OF THE CENTRAL NERVOUS SYSTEM AND PERIPHERAL ORGANS OF THE FRESHWATER SNAIL PLANORBARIUS CORNEUS

1996 ◽  
Vol 28 (4) ◽  
pp. 417-424 ◽  
Author(s):  
MARIA ENRICA FERRETTI ◽  
DARIO SONETTI ◽  
MARIA CRISTINA PARESCHI ◽  
MARCO BUZZI ◽  
MARIA LUISA COLAMUSSI ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Adenylate Cyclase ◽  
Freshwater Snail ◽  
Planorbarius Corneus ◽  
Peripheral Organs ◽  
The Central Nervous System
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