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Interorgan amino acid interchange in propionic acidemia: the missing key to understanding its physiopathology
BACKGROUND: Propionic acidemia is an inborn error of metabolism caused by a deficiency in the mitochondrial enzyme propionyl-CoA carboxylase that converts the propionyl CoA to methyl malonyl CoA. This leads to profound changes in distinct metabolic pathways, including the urea cycle, with consequenc...
| Autores principales: | , , , , , , , |
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| Formato: | Online Artículo Texto |
| Lenguaje: | English |
| Publicado: |
Springer Vienna
2022
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| Materias: | |
| Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9167193/ https://www.ncbi.nlm.nih.gov/pubmed/35098378 http://dx.doi.org/10.1007/s00726-022-03128-6 |