Evidence map›Paper›PMID 36777637›Full record

ArticleFrontiers in neuroscience2023

A new FRDA mouse model [

Ester Kalef-Ezra, Fred Jonathan Edzeamey, Adamo Valle, Hassan Khonsari, Paula Kleine, Carlo Oggianu, Sahar Al-Mahdawi, Mark A Pook, Sara Anjomani Virmouni

Abstract read
In one paragraph

Article in Frontiers in neuroscience, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.

0numbers the graph read from it
0cells of the map it votes in
14citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

14 citing papers in PubMed.

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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

9 authors.

Ester Kalef-EzraAtaxia Research Group, Division of Biosciences, Department of Life Sciences, Brunel University London, Uxbridge, United Kingdom.
Fred Jonathan EdzeameyAtaxia Research Group, Division of Biosciences, Department of Life Sciences, Brunel University London, Uxbridge, United Kingdom.
Adamo ValleEnergy Metabolism and Nutrition, Research Institute of Health Sciences (IUNICS), University of Balearic Islands, Palma, Spain.
Hassan KhonsariAtaxia Research Group, Division of Biosciences, Department of Life Sciences, Brunel University London, Uxbridge, United Kingdom.
Paula KleineAtaxia Research Group, Division of Biosciences, Department of Life Sciences, Brunel University London, Uxbridge, United Kingdom.
Carlo OggianuAtaxia Research Group, Division of Biosciences, Department of Life Sciences, Brunel University London, Uxbridge, United Kingdom.
Sahar Al-MahdawiAtaxia Research Group, Division of Biosciences, Department of Life Sciences, Brunel University London, Uxbridge, United Kingdom.
Mark A PookAtaxia Research Group, Division of Biosciences, Department of Life Sciences, Brunel University London, Uxbridge, United Kingdom.
Sara Anjomani VirmouniAtaxia Research Group, Division of Biosciences, Department of Life Sciences, Brunel University London, Uxbridge, United Kingdom.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Introduction: Friedreich's ataxia (FRDA) is an inherited recessive neurodegenerative disorder caused by a homozygous guanine-adenine-adenine (GAA) repeat expansion within intron 1 of the Methods: We obtained the latest FRDA humanized mouse model that was generated on the basis of our previous YG8sR, by Jackson laboratory [YG8JR, Results: We found statistically significant behavioral deficits, together with reduced levels of frataxin mRNA and protein, and aconitase activity in YG8JR mice compared with control Y47JR mice. YG8JR mice exhibit intergenerational GAA repeat instability by the analysis of parent and offspring tissue samples. Somatic GAA repeat instability was also detected in individual brain and cerebellum tissue samples. In addition, increased DNA methylation of CpG U13 was identified in Discussion: These studies provide a detailed characterization of the GAA repeat expansion-based YG8JR transgenic mouse models that will help investigations of FRDA disease mechanisms and therapy.

Indexed as

frataxinFRDAFriedreich’s ataxiaFXNGAA repeatmouse modelY47JRYG8JR

Identifiers

PMID36777637
PMCPMC9909538

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.