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Article

Renal phenotyping in a hypomorphic murine model of propionic aciduria reveals common pathomechanisms in organic acidurias.

Scientific reports - 16 Dec 2024

Schumann Anke, Martinez-Pizarro Ainhoa, Richard Eva, Schell Christoph, Kössinger Anna Laura, Zeyer Karina A, Tholen Stefan, Schilling Oliver, Barry Michael, Neubauer Björn, Köttgen Michael, Hannibal Luciana, Desviat Lourdes R, Spiekerkötter Ute

Abstract excerpt

Mutations in the mitochondrial enzyme propionyl-CoA carboxylase (PCC) cause propionic aciduria (PA). Chronic kidney disease (CKD) is a known long-term complication. However, good metabolic control and standard therapy fail to prevent CKD. The pathophysiological mechanisms of CKD are unclear. We investigated the renal phenotype of a hypomorphic murine PA model (Pcca-/-(A138T)) to identify CKD-driving mechanisms....

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Citric Acid Cycle
Kidney
Methylmalonyl-CoA Decarboxylase
Mitochondrial Dynamics

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Oxidative Stress

Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha

Renal Insufficiency, Chronic

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Identifiers

PMID: 39681572
DOI: 10.1038/s41598-024-79572-z
PMCID: PMC11649940

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