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The transmissible brain amyloidoses: a comparison with the non transmissible brain amyloidoses of Alzheimer type. 1993

P P Liberski
Department of Oncology, Medical Academy, Lódź, Poland.

I report here the basic concepts of the transmissible and non-transmissible cerebral amyloidoses. The pathogenesis of both types of brain amyloidoses consist of a synthesis of amyloid precursor followed by its processing to yield a final postranslationally modified deposit. PrPc and APP serve as precursor proteins while modified PrP (PrPsc and PrP 27-30) and beta-A4 as final deposits in transmissible and non-transmissible brain amyloidoses, respectively. The hallmark of both types of brain amyloidoses is amyloid deposits immunoreactive to appropriate amyloids. Other, mostly unspecific, neuropathological phenomena as neurofibrillary tangles, dystrophic neurites and granulovacuolar degeneration are also discussed. The only disease-specific structure for transmissible cerebral amyloidoses are tubulovesicular structures of unknown biological significance. They may even be the infectious virus.

UI MeSH Term Description Entries
D001921 Brain The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM. Encephalon
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000544 Alzheimer Disease A degenerative disease of the BRAIN characterized by the insidious onset of DEMENTIA. Impairment of MEMORY, judgment, attention span, and problem solving skills are followed by severe APRAXIAS and a global loss of cognitive abilities. The condition primarily occurs after age 60, and is marked pathologically by severe cortical atrophy and the triad of SENILE PLAQUES; NEUROFIBRILLARY TANGLES; and NEUROPIL THREADS. (From Adams et al., Principles of Neurology, 6th ed, pp1049-57) Acute Confusional Senile Dementia,Alzheimer's Diseases,Dementia, Alzheimer Type,Dementia, Senile,Presenile Alzheimer Dementia,Senile Dementia, Alzheimer Type,Alzheimer Dementia,Alzheimer Disease, Early Onset,Alzheimer Disease, Late Onset,Alzheimer Sclerosis,Alzheimer Syndrome,Alzheimer Type Senile Dementia,Alzheimer's Disease,Alzheimer's Disease, Focal Onset,Alzheimer-Type Dementia (ATD),Dementia, Presenile,Dementia, Primary Senile Degenerative,Early Onset Alzheimer Disease,Familial Alzheimer Disease (FAD),Focal Onset Alzheimer's Disease,Late Onset Alzheimer Disease,Primary Senile Degenerative Dementia,Senile Dementia, Acute Confusional,Alzheimer Dementias,Alzheimer Disease, Familial (FAD),Alzheimer Diseases,Alzheimer Type Dementia,Alzheimer Type Dementia (ATD),Alzheimers Diseases,Dementia, Alzheimer,Dementia, Alzheimer-Type (ATD),Familial Alzheimer Diseases (FAD),Presenile Dementia,Sclerosis, Alzheimer,Senile Dementia
D000686 Amyloidosis A group of sporadic, familial and/or inherited, degenerative, and infectious disease processes, linked by the common theme of abnormal protein folding and deposition of AMYLOID. As the amyloid deposits enlarge they displace normal tissue structures, causing disruption of function. Various signs and symptoms depend on the location and size of the deposits. Amyloidoses
D017096 Prion Diseases A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA; ATAXIA; and a fatal outcome. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The older literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95(23):13363-83) Dementias, Transmissible,Spongiform Encephalopathies, Transmissible,Transmissible Dementias,Encephalopathies, Spongiform, Transmissible,Human Transmissible Spongiform Encephalopathies, Inherited,Inherited Human Transmissible Spongiform Encephalopathies,Prion Disease,Prion Protein Diseases,Prion-Associated Disorders,Prion-Induced Disorder,Prion-Induced Disorders,Transmissible Spongiform Encephalopathies,Dementia, Transmissible,Disorder, Prion-Induced,Disorders, Prion-Induced,Encephalopathies, Transmissible Spongiform,Encephalopathy, Transmissible Spongiform,Prion Induced Disorder,Prion Protein Disease,Spongiform Encephalopathy, Transmissible,Transmissible Dementia,Transmissible Spongiform Encephalopathy

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