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Metabolic adaptation allows Amacr-deficient mice to remain symptom-free despite low levels of mature bile acids.
Selkälä EM, Kuusisto SM, Salonurmi T, Savolainen MJ, Jauhiainen M, Pirilä PL, Kvist AP, Conzelmann E, Schmitz W, Alexson SE, Kotti TJ, Hiltunen JK, Autio KJ. Selkälä EM, et al. Among authors: conzelmann e. Biochim Biophys Acta. 2013 Aug;1831(8):1335-43. doi: 10.1016/j.bbalip.2013.05.002. Epub 2013 May 13. Biochim Biophys Acta. 2013. PMID: 23680781
Mitochondrial 2,4-dienoyl-CoA reductase deficiency in mice results in severe hypoglycemia with stress intolerance and unimpaired ketogenesis.
Miinalainen IJ, Schmitz W, Huotari A, Autio KJ, Soininen R, Ver Loren van Themaat E, Baes M, Herzig KH, Conzelmann E, Hiltunen JK. Miinalainen IJ, et al. Among authors: conzelmann e. PLoS Genet. 2009 Jul;5(7):e1000543. doi: 10.1371/journal.pgen.1000543. Epub 2009 Jul 3. PLoS Genet. 2009. PMID: 19578400 Free PMC article.
The role of alpha-methylacyl-CoA racemase in bile acid synthesis.
Cuebas DA, Phillips C, Schmitz W, Conzelmann E, Novikov DK. Cuebas DA, et al. Among authors: conzelmann e. Biochem J. 2002 May 1;363(Pt 3):801-7. doi: 10.1042/0264-6021:3630801. Biochem J. 2002. PMID: 11964182 Free PMC article.
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