Dataset Information

Long-Term Benefits of TUDCA Supplement in ARSACS Zebrafish Model

ABSTRACT: Our work demonstrated an impairment in cognitive, social and emotional behavior in aged sacs knockout zebrafish similar to that observed in human patients. Transcriptomic and proteomic data from the adult brains revealing alterations in several genes related to circadian rhythms and neuroinflammation in adult mutants. t Interestingly, alterations in sleep and circadian rhythms are common in individuals with cerebellar ataxia and may contribute to cognitive function and behavior. Long term treatment with TUDCA , a neuroprotective molecule,partially rescued social and cognitive deficits. These findings suggest the potential for further optimization of this neuroprotective compound in subsequent preclinical and clinical studies.

INSTRUMENT(S):

ORGANISM(S): Danio Rerio (zebrafish) (brachydanio Rerio)

TISSUE(S): Brain

SUBMITTER: Gabriela Coronel Vargas

LAB HEAD: Valentina Naef

PROVIDER: PXD058522 | Pride | 2025-09-03

REPOSITORIES: Pride

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Dataset's files

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			Action	DRS
	2024_09_30_proj_06_neuroDR_adultbrain_sp_without_sacslosslosstudca.mzTab	Mztab
	2024_09_30_proj_06_neuroDR_adultbrain_sp_without_sacslosslosstudca_Proteins.txt	Txt
	2024_10_11_proj_06_neuroDR_adultbrain_sp_only_sacslosslosstudca.mzTab	Mztab
	2024_10_11_proj_06_neuroDR_adultbrain_sp_only_sacslosslosstudca_Proteins.txt	Txt
	proj_06_neuroDR_Adult_DepositedSamples.csv	Csv

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Long-term benefits of TUDCA supplement in ARSACS zebrafish model.

Naef Valentina V Vecchia Stefania Della SD Giacich Michela M Licitra Rosario R Bachetti Tiziana T Vargas Gabriela Coronel GC Ponassi Marco M Santorelli Filippo Maria FM

Scientific reports 20250714 1

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is an early-onset neurodevelopmental and neurodegenerative disorder characterized by ataxia, spasticity, and peripheral neuropathy. However, several studies have highlighted that some patients also experience cognitive, emotional and social deficits, suggesting a more complex clinical picture that extends beyond motor symptoms. Building on these findings, this study aimed to: (i) investigate locomotor, social and cognitive defici ...[more]

PMID: 40659819

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