Hepatic hydrogen sulfide levels are reduced in mouse model of Hutchinson-Gilford progeria syndrome.

ABSTRACT: Hutchinson-Gilford progeria syndrome (HGPS) is a rare human disease characterised by accelerated biological ageing. Current treatments are limited, and most patients die before 15 years of age. Hydrogen sulfide (H₂S) is an important gaseous signalling molecule that it central to multiple cellular homeostasis mechanisms. Dysregulation of tissue H₂S levels is thought to contribute to an ageing phenotype in many tissues across animal models. Whether H₂S is altered in HGPS is unknown. We investigated hepatic H₂S production capacity and transcript, protein and enzymatic activity of proteins that regulate hepatic H₂S production and disposal in a mouse model of HGPS (G609G mice, mutated Lmna gene equivalent to a causative mutation in HGPS pat

SUBMITTER: Wilkie SE 

PROVIDER: S-EPMC10333079 | biostudies-literature | 2023 Jun

REPOSITORIES: biostudies-literature