Evidence map›Paper›PMID 29563343›Full record

ArticleJCI insight2018

Neuropathic pain in a Fabry disease rat model.

James J Miller, Kazuhiro Aoki, Francie Moehring, Carly A Murphy, Crystal L O'Hara, Michael Tiemeyer, Cheryl L Stucky, Nancy M Dahms

Open access · goldAbstract read
In one paragraph

Article in JCI insight, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 49 papers.

0numbers the graph read from it
0cells of the map it votes in
49citing papers in PubMed
4.4field-weighted citation impact, top 5% of its field
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

49 citing papers in PubMed, 69 citations in OpenAlex.

  1. Article
  2. Fabry Disease: A Focus on the Role of Oxidative Stress.Antioxidants (Basel, Switzerland) · 2026
    Review
  3. Not Just Neurons: Pain Is Orchestrated in Partnership with Many Non-neuronal Cells.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2025
    Review
  4. Article
  5. Article
  6. Article
  7. Article
  8. Article
  9. Review
  10. Review
  11. Article
  12. Review
  13. Article
  14. Article
  15. Article
  16. Article
  17. Article
  18. Review
  19. Article
  20. Article
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

8 authors at 2 institutions in 1 country.

James J MillerDepartment of Biochemistry, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Kazuhiro AokiComplex Carbohydrate Research Center, University of Georgia, Athens, Georgia, USA.
Francie MoehringDepartment of Cell Biology, Neurobiology, & Anatomy, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Carly A MurphyDepartment of Biochemistry, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Crystal L O'HaraDepartment of Cell Biology, Neurobiology, & Anatomy, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Michael TiemeyerComplex Carbohydrate Research Center, University of Georgia, Athens, Georgia, USA.
Cheryl L StuckyDepartment of Cell Biology, Neurobiology, & Anatomy, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Nancy M DahmsDepartment of Biochemistry, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Medical College of Wisconsin · USUniversity of Georgia · US

Funding

TR&D1: Stem Cell and Induced Pluripotent Stem Cell Resources (Pages 116-134)P41GM103490 · NIGMS · UNIVERSITY OF GEORGIA · PI PIERCE, J. MICHAEL · 2012 to 2018
$13.7M
STRUCTURAL ANALYSIS OF THE MANNOSE 6-PHOSPHATE RECEPTORSR01DK042667 · NIDDK · MEDICAL COLLEGE OF WISCONSIN · PI DAHMS, NANCY M. · 1997 to 2023
$9.8M
Nociceptive Mechanisms Underlying Sickle Cell PainR01NS070711 · NINDS · MEDICAL COLLEGE OF WISCONSIN · PI Bonnie N Dittel, Cheryl Louise Stucky · 2009 to 2026
$8.7M
Gene targeted rat resource for the study of complex diseaseR24HL114474 · NHLBI · MEDICAL COLLEGE OF WISCONSIN · PI DWINELL, MELINDA R · 2013 to 2017
$8.7M
FUNCTIONAL ANALYSIS OF DISTINCT NOCICEPTOR POPULATIONSR01NS040538 · NINDS · MEDICAL COLLEGE OF WISCONSIN · PI STUCKY, CHERYL LOUISE · 2000 to 2019
$6.2M
Medical Scientist Training ProgramT32GM080202 · NIGMS · MEDICAL COLLEGE OF WISCONSIN · PI BARBIERI, JOSEPH T, SALZMAN, NITA H · 2010 to 2024
$5.7M
Development of high-throughput workflow for glycosphingolipid analysis and annotationR21AI129873 · NIAID · UNIVERSITY OF GEORGIA · PI AOKI, KAZUHIRO, RANZINGER, RENE · 2016 to 2017
$582k
Fabry Disease in the Gla Knockout Rat: Development of Novel Protein TherapeuticsR21NS095627 · NINDS · MEDICAL COLLEGE OF WISCONSIN · PI DAHMS, NANCY M. · 2015 to 2016
$369k
Fabry Disease: Characterization of a Rat Model and Modification of a Therapeutic ProteinF30DK113641 · NIDDK · MEDICAL COLLEGE OF WISCONSIN · PI MILLER, JAMES J · 2016 to 2019
$185k
NHLBI NIH HHS R24 HL114474NIAID NIH HHS R21 AI129873NIDDK NIH HHS F30 DK113641NIDDK NIH HHS R01 DK042667NIGMS NIH HHS P41 GM103490NIGMS NIH HHS T32 GM080202NINDS NIH HHS R01 NS040538NINDS NIH HHS R01 NS070711NINDS NIH HHS R21 NS095627
6 · The paper itself

Abstract

Fabry disease, the most common lysosomal storage disease, affects multiple organs and results in a shortened life span. This disease is caused by a deficiency of the lysosomal enzyme α-galactosidase A, which leads to glycosphingolipid accumulation in many cell types. Neuropathic pain is an early and severely debilitating symptom in patients with Fabry disease, but the cellular and molecular mechanisms that cause the pain are unknown. We generated a rat model of Fabry disease, the first nonmouse model to our knowledge. Fabry rats had substantial serum and tissue accumulation of α-galactosyl glycosphingolipids and had pronounced mechanical pain behavior. Additionally, Fabry rat dorsal root ganglia displayed global N-glycan alterations, sensory neurons were laden with inclusions, and sensory neuron somata exhibited prominent sensitization to mechanical force. We found that the cation channel transient receptor potential ankyrin 1 (TRPA1) is sensitized in Fabry rat sensory neurons and that TRPA1 antagonism reversed the behavioral mechanical sensitization. This study points toward TRPA1 as a potentially novel target to treat the pain experienced by patients with Fabry disease.

Indexed as

alpha-GalactosidaseAnimalsAnimals, Genetically ModifiedBehavior, AnimalDisease Models, AnimalElectrophysiologyFabry DiseaseFemaleGanglia, SpinalGene Knockdown TechniquesGenetic Predisposition to DiseaseGlycosphingolipidsHumansLiverMaleNeuralgiaalpha-GalactosidaseGLA protein, humanGlycosphingolipidsTRPA1 Cation ChannelTrpa1 protein, ratGenetic diseasesGlycobiologyNeurosciencePain

Identifiers

PMID29563343
PMCPMC5926911
OpenAlexW2791242127

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.