Ortiz, G.G.; Ramírez-Jirano, J.; Arizaga, R.L.; Delgado-Lara, D.L.C.; Torres-Sánchez, E.D. Frontotemporal-TDP and LATE Neurocognitive Disorders: A Pathophysiological and Genetic Approach. Brain Sci.2023, 13, 1474.
Ortiz, G.G.; Ramírez-Jirano, J.; Arizaga, R.L.; Delgado-Lara, D.L.C.; Torres-Sánchez, E.D. Frontotemporal-TDP and LATE Neurocognitive Disorders: A Pathophysiological and Genetic Approach. Brain Sci. 2023, 13, 1474.
Ortiz, G.G.; Ramírez-Jirano, J.; Arizaga, R.L.; Delgado-Lara, D.L.C.; Torres-Sánchez, E.D. Frontotemporal-TDP and LATE Neurocognitive Disorders: A Pathophysiological and Genetic Approach. Brain Sci.2023, 13, 1474.
Ortiz, G.G.; Ramírez-Jirano, J.; Arizaga, R.L.; Delgado-Lara, D.L.C.; Torres-Sánchez, E.D. Frontotemporal-TDP and LATE Neurocognitive Disorders: A Pathophysiological and Genetic Approach. Brain Sci. 2023, 13, 1474.
Abstract
Frontotemporal lobar degeneration (FTLD) belongs to a heterogeneous group of highly complex neurodegenerative diseases and represents the second cause of presenile dementia in individuals under 65. Frontotemporal-TDP is a subgroup of frontotemporal dementia characterized by the aggregation of abnormal protein deposits, predominantly transactive response DNA-binding protein 43 (TDP-43), in the frontal and temporal brain regions. These deposits lead to progressive degeneration of neurons resulting in cognitive and behavioral impairments. Limbic age-related encephalopathy (LATE) pertains to age-related cognitive decline primarily affecting the limbic system which is crucial for memory, emotions, and learning. Though distinct, emerging research suggests a potential overlap in pathogenic processes, with some cases of limbic encephalopathy displaying TDP-43 pathology. Genetic factors play a pivotal role in both disorders. Mutations in various genes, such as progranulin (GRN) and chromosome 9 open reading frame 72 (C9orf72), have been identified as causative in frontotemporal-TDP. Similarly, specific genetic variants have been associated with an increased risk of developing LATE. Understanding these genetic links provides crucial insights into disease mechanisms and the potential for targeted therapies.
Keywords
frontotemporal lobar degeneration; transactive response DNA‐binding protein 43; limbic age related encephalopathy; progranulin
Subject
Medicine and Pharmacology, Neuroscience and Neurology
Copyright:
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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