Episodic pain in Fabry disease is mediated by a heat shock protein-transient receptor potential cation channel A1 axis.

ABSTRACT:

Abstract

Two-thirds of patients with Fabry disease suffer debilitating pain attacks triggered by exercise, fever, and exposure to environmental heat. These patients face an even greater risk of heat-related episodic pain in the face of global climate change. Almost nothing is known about the biological mechanisms underlying heat-induced pain crises in Fabry disease, and there is no preclinical model available to study Fabry crises. Here, we established the first model of heat-induced pain attacks in Fabry disease by exposing transgenic Fabry rats to environmental heat. Heat exposure precipitated robust mechanical hypersensitivity, closely matching temporal features reported by patients with Fabry disease. At the cellular level, heat exposure sensitized Fabry dorsal root ganglia neu

SUBMITTER: Enders JD 

PROVIDER: S-EPMC12527330 | biostudies-literature | 2026 Feb

REPOSITORIES: biostudies-literature