Glutaric aciduria type I and methylmalonic aciduria: simulation of cerebral import and export of accumulating neurotoxic dicarboxylic acids in in vitro models of the blood-brain barrier and the choroid plexus.
Sauer
Sven W
SW
Department of General Pediatrics, Division of Inborn Metabolic Diseases, University Children's Hospital Heidelberg, D-69120 Heidelberg, Germany. Sven.Sauer@med.uni-heidelberg.de
Opp
Silvana
S
Mahringer
Anne
A
Kamiński
Marcin M
MM
Thiel
Christian
C
Okun
Jürgen G
JG
Fricker
Gert
G
Morath
Marina A
MA
Kölker
Stefan
S
D000818:Animals; D001483:Base Sequence; D001812:Blood-Brain Barrier / Q000502:physiology*; D001921:Brain / Q000378:metabolism*; D002478:Cells, Cultured; D002831:Choroid Plexus / Q000378:metabolism*; D017931:DNA Primers / Q000235:genetics; D029382:Dicarboxylic Acid Transporters / Q000235:genetics / Q000378:metabolism; D003998:Dicarboxylic Acids / Q000378:metabolism*; D005977:Glutarates / Q000652:urine*; D050770:Glutaryl-CoA Dehydrogenase / Q000172:deficiency; D006801:Humans; D066298:In Vitro Techniques; D008661:Metabolism, Inborn Errors / Q000378:metabolism; D008764:Methylmalonic Acid / Q000652:urine*; D008765:Methylmalonyl-CoA Mutase / Q000172:deficiency; D008954:Models, Biological; D009498:Neurotoxins / Q000378:metabolism; D012333:RNA, Messenger / Q000235:genetics / Q000378:metabolism; D013552:Swine
D017931:DNA Primers; D029382:Dicarboxylic Acid Transporters; D003998:Dicarboxylic Acids; D005977:Glutarates; D009498:Neurotoxins; D012333:RNA, Messenger; D008764:Methylmalonic Acid; D050770:Glutaryl-CoA Dehydrogenase; D008765:Methylmalonyl-CoA Mutase; C035736:glutaric acid