Transport and distribution of 3-hydroxyglutaric acid before and during induced encephalopathic crises in a mouse model of glutaric aciduria type 1

Biochimica et Biophysica Acta - Molecular Basis of Disease

Volumn 1782, Issue 6, 2008, Pages 385-390

  Keyser, Britta ^a   Glatzel, Markus ^a   Stellmer, Franziska ^a   Kortmann, Bastian ^a   Lukacs, Zoltan ^a   Kölker, Stefan ^b   Sauer, Sven W ^b   Muschol, Nicole ^a   Herdering, Wilhelm ^c   Thiem, Joachim ^c   Goodman, Stephen I ^d   Koeller, David M ^e   Ullrich, Kurt ^a   Braulke, Thomas ^a   Mühlhausen, Chris ^a  

^a UNIVERSITY MEDICAL CENTER HAMBURG EPPENDORF   (Germany)

^b UNIVERSITY HOSPITAL HEIDELBERG   (Germany)

^c UNIVERSITY OF HAMBURG   (Germany)

^d UNIVERSITY OF COLORADO   (United States)

^e OREGON HEALTH AND SCIENCE UNIVERSITY   (United States)

Author keywords

Glutaric aciduria type 1; Glutaryl CoA dehydrogenase deficiency; Metabolic crisis; Metabolite distribution; Mouse model; Radiolabeled metabolite

Indexed keywords

3 HYDROXYGLUTARIC ACID; GLUTARIC ACID DERIVATIVE; TRITIUM;

ACIDURIA; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BODY FLUID; BRAIN DISEASE; CONTROLLED STUDY; ENDOTHELIUM CELL; ISOTOPE LABELING; MOUSE; NONHUMAN; PRIORITY JOURNAL; PROTEIN DIET; RADIOACTIVITY; TISSUE DISTRIBUTION;

AMINO ACID METABOLISM, INBORN ERRORS; ANIMALS; BIOLOGICAL TRANSPORT; BRAIN; BRAIN DISEASES; DEXTRANS; DISEASE MODELS, ANIMAL; GLUTARATES; GLUTARYL-COA DEHYDROGENASE; HUMANS; IMMUNOHISTOCHEMISTRY; INTESTINES; KIDNEY; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT;

MUS;

EID: 43549096394     PISSN: 09254439     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.bbadis.2008.02.008     Document Type: Article

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