Aldolase B Deficient Mice Are Characterized by Hepatic Nucleotide Sugar Abnormalities.

ABSTRACT: Hereditary fructose intolerance (HFI) is characterized by liver damage and a secondary defect in N-linked glycosylation due to impairment of mannose phosphate isomerase (MPI). Mannose treatment has been shown to be an effective treatment in a primary defect in MPI (i.e., MPI-CDG), which is also characterized by liver damage. Therefore, the aims of this study were to determine: (1) hepatic nucleotide sugar levels, and (2), the effect of mannose supplementation on hepatic nucleotide sugar levels and liver fat, in a mouse model for HFI. Aldolase B deficient mice (Aldob^-/-) were treated for four weeks with 5% mannose via the drinking water and compared to Aldob^-/- mice and wildtype mice treated with regular drinking water. We found that hepatic GDP-mannose and hepatic GDP

SUBMITTER: Buziau AM 

PROVIDER: S-EPMC11672228 | biostudies-literature | 2025 Jan

REPOSITORIES: biostudies-literature