Evidence map›Paper›PMID 42402290›Full record

ArticleBehavioural brain research2026

Behavioral characterization of bulbar sensorimotor function in a rat model of Alexander disease.

Jared S Cullen, John A Russell, Cynthia A Kelm-Nelson, John C Szot, Albee Messing, Tracy L Hagemann, Nadine P Connor

Erratum issuedAbstract read
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In one paragraph

Article in Behavioural brain research, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing 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

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

7 authors.

Jared S CullenDepartment of Otolaryngology-Head and Neck Surgery, University of Wisconsin-Madison, Madison, WI, USA. Electronic address: jared.cullen@wisc.edu.
John A RussellDepartment of Otolaryngology-Head and Neck Surgery, University of Wisconsin-Madison, Madison, WI, USA.
Cynthia A Kelm-NelsonDepartment of Surgery-Endocrine, University of Wisconsin-Madison, Madison, WI, USA.
John C SzotDepartment of Otolaryngology-Head and Neck Surgery, University of Wisconsin-Madison, Madison, WI, USA.
Albee MessingDepartment of Comparative Biosciences, University of Wisconsin-Madison, Madison, WI, USA; Waisman Center, University of Wisconsin-Madison, Madison, WI, USA.
Tracy L HagemannWaisman Center, University of Wisconsin-Madison, Madison, WI, USA.
Nadine P ConnorDepartment of Otolaryngology-Head and Neck Surgery, University of Wisconsin-Madison, Madison, WI, USA.

Funding

University of Wisconsin Voice Research Training Program (T32)T32DC009401 · NIDCD · UNIVERSITY OF WISCONSIN-MADISON · PI Susan Lynn Thibeault · 2009 to 2026
$6.3M
Waisman Intellectual and Developmental Disabilities Research Center Down Syndrome RegistryU54HD090256 · NICHD · UNIVERSITY OF WISCONSIN-MADISON · PI MESSING, ALBEE · 2016 to 2020
$6.0M
Patient-derived iPS cells as model systems for AxDP01HD076892 · NICHD · UNIVERSITY OF WISCONSIN-MADISON · PI NEDERGAARD, MAIKEN · 2014 to 2018
$6.0M
Mechanisms of swallowing dysfunction and rescue in a translational rat model of Alzheimer's diseaseR01AG085564 · NIA · UNIVERSITY OF WISCONSIN-MADISON · PI NADINE P CONNOR, Michelle Renee Ciucci · 2023 to 2026
$3.1M
Pathways, mechanisms, and treatments of vocal communication deficits in a Parkinson rat modelR01NS117469 · NINDS · UNIVERSITY OF WISCONSIN-MADISON · PI Cynthia A Kelm-Nelson · 2020 to 2026
$3.0M
Bioenergetic Mechanisms of Tongue Muscle FatigueR01DC018071 · NIDCD · UNIVERSITY OF WISCONSIN-MADISON · PI CONNOR, NADINE P · 2019 to 2023
$2.5M
Treatment of Functional Deficits IN tongue muscles induced by radiation and chemoradiation treatmentR37CA225608 · NCI · UNIVERSITY OF WISCONSIN-MADISON · PI RUSSELL, JOHN A · 2018 to 2024
$2.4M
NCI NIH HHS R37 CA225608NIA NIH HHS R01 AG085564NICHD NIH HHS P01 HD076892NICHD NIH HHS U54 HD090256NIDCD NIH HHS R01 DC018071NIDCD NIH HHS T32 DC009401NINDS NIH HHS R01 NS117469
6 · The paper itself

Abstract

Alexander disease (AxD) is a neurodegenerative disorder caused by mutations in the glial fibrillary acidic protein (GFAP) gene and is associated with bulbar sensorimotor impairments including dysphagia, dysphonia, and dysarthria. This study assessed oromotor and laryngeal function in the R237H rat model of AxD compared with wild-type (WT) controls. Rats underwent behavioral assessments of tongue strength and press rate, mastication, swallowing, and ultrasonic vocalizations. Compared with WT controls, the R237H group exhibited significantly reduced tongue force and press rates, with a decline in performance over time, indicating lingual weakness and reduced capacity for sustained performance. During mastication, the R237H group required longer consumption times and more chewing events despite similar bite counts, reflecting inefficient oral processing. Videofluoroscopic swallow studies revealed prolonged oral-phase duration and an increased number of jaw cycles prior to swallow initiation in the R237H group, suggesting impaired oromotor coordination during swallow, while bolus area, jaw cycle rate, and bolus velocity were preserved. Ultrasonic vocalization analyses revealed shorter durations among the longest calls in the R237H group, suggesting reduced vocal performance. Collectively, these findings demonstrate that the R237H rat model of AxD exhibits significant deficits in tongue function, mastication, swallowing, and vocalization. These behavioral impairments are consistent with bulbar symptoms observed in individuals with AxD and extend prior characterization of this model. This study provides a behavioral foundation for future mechanistic and translational investigations of bulbar dysfunction in this model.

Indexed as

Alexander DiseaseAnimalsDeglutitionDisease Models, AnimalGlial Fibrillary Acidic ProteinMaleMasticationRatsTongueVocalization, AnimalGlial Fibrillary Acidic ProteinAlexander diseaseAnimal behaviorBulbar dysfunctionGlial fibrillary acidic proteinOromotor dysfunctionRat model

Identifiers

PMID42402290
PMCPMC13502299

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

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.