Understanding cerebral L-lysine metabolism: the role of L-pipecolate metabolism in Gcdh-deficient mice as a model for glutaric aciduria type I View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2015-03

AUTHORS

Roland Posset, Silvana Opp, Eduard A. Struys, Alfred Völkl, Heribert Mohr, Georg F. Hoffmann, Stefan Kölker, Sven W. Sauer, Jürgen G. Okun

ABSTRACT

Inherited deficiencies of the L-lysine catabolic pathway cause glutaric aciduria type I and pyridoxine-dependent epilepsy. Dietary modulation of cerebral L-lysine metabolism is thought to be an important therapeutic intervention for these diseases. To better understand cerebral L-lysine degradation, we studied in mice the two known catabolic routes -- pipecolate and saccharopine pathways -- using labeled stable L-lysine and brain peroxisomes purified according to a newly established protocol. Experiments with labeled stable L-lysine show that cerebral L-pipecolate is generated along two pathways: i) a minor proportion retrograde after ε-deamination of L-lysine along the saccharopine pathway, and ii) a major proportion anterograde after α-deamination of L-lysine along the pipecolate pathway. In line with these findings, we observed only little production of saccharopine in the murine brain. L-pipecolate oxidation was only detectable in brain peroxisomes, but L-pipecolate oxidase activity was low (7 ± 2μU/mg protein). In conclusion, L-pipecolate is a major degradation product from L-lysine in murine brain generated by α-deamination of this amino acid. More... »

PAGES

265-272

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s10545-014-9762-z

DOI

http://dx.doi.org/10.1007/s10545-014-9762-z

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1043619266

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/25214427


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