Normal Urinary Oxalate Excretion in 4-Hydroxy-2-Oxo-Glutarate Aldolase 1 (HOGA1) Deficient Mice With Agt Expression in Peroxisomes and Not in Mitochondria

Authors

• Iolanda Boffa
• Rosa Ferriero
• Mariarosaria Cancelliere
• Edoardo Nusco
• Leonardo Gatticchi
• Donna Palmer
• Philip Ng
• Pasquale Piccolo
• Barbara Cellini
• Kyle Wood
• John Knight
• Nicola Brunetti-Pierri

Language

English

Publication Date

10-1-2025

Journal

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease

DOI

10.1016/j.bbadis.2025.167963

PMID

40578401

PMCID

PMC13238155

PubMedCentral® Full Text Version

Author MSS

Abstract

Primary hyperoxaluria type 3 (PH3) is caused by mutations in Hoga1 gene. PH3 individuals develop nephrolithiasis, but the mechanism underlying hyperoxaluria is unclear and a mouse model recapitulating the human disease can provide insights into the pathogenesis of PH3. Hoga1−/− mice do not have increased urinary oxalate excretion, probably due to the murine mitochondrial alanine-glioxylate-aminotransferase (AGT) activity, which in humans is only expressed in peroxisomes. However, Hoga1−/−/Agxt−/− mice with AGT installed on peroxisome and not on mitochondria did not show increased urinary oxalate, suggesting that AGT expression in both cellular compartments is not an explanation for the lack of hyperoxaluria in Hoga1−/− mice.

Keywords

Animals, Peroxisomes, Mice, Mitochondria, Oxalates, Mice, Knockout, Transaminases, Oxo-Acid-Lyases, Hyperoxaluria, Primary, Humans, Primary Hyperoxaluria Type 3, 4-Hydroxy-2-oxo-glutarate aldolase, HOGA1

Published Open-Access

yes

Recommended Citation

Boffa, Iolanda; Ferriero, Rosa; Cancelliere, Mariarosaria; et al., "Normal Urinary Oxalate Excretion in 4-Hydroxy-2-Oxo-Glutarate Aldolase 1 (HOGA1) Deficient Mice With Agt Expression in Peroxisomes and Not in Mitochondria" (2025). Faculty, Staff and Students Publications. 7155.
https://digitalcommons.library.tmc.edu/baylor_docs/7155