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Article

Multifactorial modulation of susceptibility to l-lysine in an animal model of glutaric aciduria type I.

Biochimica et biophysica acta - 1 May 2015

Sauer Sven W, Opp Silvana, Komatsuzaki Shoko, Blank Anna-Eva, Mittelbronn Michel, Burgard Peter, Koeller D M, Okun Jürgen G, Kölker Stefan

Abstract excerpt

Glutaric aciduria type I is an inherited defect in L-lysine, L-hydroxylysine and L-tryptophan degradation caused by deficiency of glutaryl-CoA dehydrogenase (GCDH). The majority of untreated patients presents with accumulation of neurotoxic metabolites - glutaric acid (GA) and 3-hydroxyglutaric acid (3-OHGA) - and striatal injury. Gcdh(-/-) mice display elevated levels of GA and 3-OH-GA but do not spontaneously...

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Source

Identifiers

PMID: 25558815
DOI: 10.1016/j.bbadis.2014.12.022

Genes

GCDH

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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