Linßen, Johannes
[VerfasserIn]
;
Sereda, Michael
[AkademischeR BetreuerIn];
Sereda, Michael
[Sonstige Person, Familie und Körperschaft];
Thoms, Sven
[Sonstige Person, Familie und Körperschaft]
Analyse der Auswirkungen des PPARα-Liganden Fenofibrat auf Abcd1-defiziente Mäuse
Beschreibung:
The X-linked adrenoleukodystrophy is a peroxisomal hereditary metabolic disease that is due to the loss of function of the peroxisomal transmembrane transporter ABCD1. We distinguish between the frequent progressive forms of infantile cerebral adrenoleukodystrophy (CALD) and the adrenomyeloneuropathy (AMN). While the AMN usually only manifests between the twentieth and thirtieth year, the CALD already in the first years of life occurs and lethal disease. Due to loss of function of the transmembrane transporter it comes to the accumulation of very long chain fatty acids and to an inflammator...