The Advance on Frontotemporal Dementia (FTD)’s Neuropathology and Molecular Genetics

Wang, Jindong; Wang, Bailing; Zhou, Tiantian

doi:10.1155/2022/5003902

Cited by 2 publications

(2 citation statements)

References 53 publications

(53 reference statements)

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“… Mandrioli et al (2019) also mention that both diseases are accompanied by neuroinflammation with inflammasomes. FTD is a group of similar threatening and rapidly progressing cognitive disorders with a rather high mortality ( Wang et al, 2022 ). The pathology comprises a storage of pathological tau, Fus, and TDP-43 with predominant harm to the neurons in the frontal and temporal cortex ( Josephs et al, 2011 ).…”

Section: Hsp70 In Ndsmentioning

confidence: 99%

Closest horizons of Hsp70 engagement to manage neurodegeneration

Venediktov,

Bushueva,

Kudryavtseva

et al. 2023

Front. Mol. Neurosci.

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Our review seeks to elucidate the current state-of-the-art in studies of 70-kilodalton-weighed heat shock proteins (Hsp70) in neurodegenerative diseases (NDs). The family has already been shown to play a crucial role in pathological aggregation for a wide spectrum of brain pathologies. However, a slender boundary between a big body of fundamental data and its implementation has only recently been crossed. Currently, we are witnessing an anticipated advancement in the domain with dozens of studies published every month. In this review, we briefly summarize scattered results regarding the role of Hsp70 in the most common NDs including Alzheimer’s disease (AD), Parkinson’s disease (PD), and amyotrophic lateral sclerosis (ALS). We also bridge translational studies and clinical trials to portray the output for medical practice. Available options to regulate Hsp70 activity in NDs are outlined, too.

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Section: Hsp70 In Ndsmentioning

confidence: 99%

Closest horizons of Hsp70 engagement to manage neurodegeneration

Venediktov,

Bushueva,

Kudryavtseva

et al. 2023

Front. Mol. Neurosci.

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“…Individuals affected by ALS exhibit an average repeat count ranging from 700 to 1600, whereas healthy individuals possess fewer than 25 repeats [23][24][25][26]. As for FTD, approximately one-third of FTDs are familial, with autosomal dominant mutations in three genes accounting for the majority of inheritance, including progranulin (GRN), C9orf72, and microtubule-associated protein tau (MAPT) [27]. The co-occurrence of these two disorders among families provides support for their genetic linkage [28].…”

Section: Introductionmentioning

confidence: 99%

Advances in the Structure of GGGGCC Repeat RNA Sequence and Its Interaction with Small Molecules and Protein Partners

Liu,

Zhao,

et al. 2023

Molecules

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The aberrant expansion of GGGGCC hexanucleotide repeats within the first intron of the C9orf72 gene represent the predominant genetic etiology underlying amyotrophic lateral sclerosis (ALS) and frontal temporal dementia (FTD). The transcribed r(GGGGCC)n RNA repeats form RNA foci, which recruit RNA binding proteins and impede their normal cellular functions, ultimately resulting in fatal neurodegenerative disorders. Furthermore, the non-canonical translation of the r(GGGGCC)n sequence can generate dipeptide repeats, which have been postulated as pathological causes. Comprehensive structural analyses of r(GGGGCC)n have unveiled its polymorphic nature, exhibiting the propensity to adopt dimeric, hairpin, or G-quadruplex conformations, all of which possess the capacity to interact with RNA binding proteins. Small molecules capable of binding to r(GGGGCC)n have been discovered and proposed as potential lead compounds for the treatment of ALS and FTD. Some of these molecules function in preventing RNA–protein interactions or impeding the phase transition of r(GGGGCC)n. In this review, we present a comprehensive summary of the recent advancements in the structural characterization of r(GGGGCC)n, its propensity to form RNA foci, and its interactions with small molecules and proteins. Specifically, we emphasize the structural diversity of r(GGGGCC)n and its influence on partner binding. Given the crucial role of r(GGGGCC)n in the pathogenesis of ALS and FTD, the primary objective of this review is to facilitate the development of therapeutic interventions targeting r(GGGGCC)n RNA.

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The Advance on Frontotemporal Dementia (FTD)’s Neuropathology and Molecular Genetics

Cited by 2 publications

References 53 publications

Closest horizons of Hsp70 engagement to manage neurodegeneration

Closest horizons of Hsp70 engagement to manage neurodegeneration

Advances in the Structure of GGGGCC Repeat RNA Sequence and Its Interaction with Small Molecules and Protein Partners

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