Purhonen 2014 Abstract MiP2014

Effect of ketogenic diet in mouse model of the mitochondrial hepatopathy GRACILE syndrome.

Link: Mitochondr Physiol Network 19.13 - MiP2014

Purhonen J, Kallijaervi J, Rajendran J, Jauhiainen M, Kotarsky H, Fellman V (2014)

Event: MiP2014

By author request, this abstract has been removed because it contains preliminary observations with a minor conflict with the final results.

• O2k-Network Lab: FI Helsinki Mervaala E

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Affiliation

1-Folkhälsan Research Center; 2-Dep Food Environm Sc, Univ Helsinki; 3-Nat Inst Health Welfare; Helsinki, Finland; 4-Dep Pediatrics, Lund Univ, Sweden; 5-Children’s Hostital, Helsinki Univ Hospital, Univ Helsinki, Finland. − janne.purhonen@helsinki.fi

References

1. Fellman V, Rapola J, Pihko H, Varilo T, Raivio KO (1998) Iron-overload disease in infants involving fetal growth retardation, lactic acidosis, liver haemosiderosis, and aminoaciduria. Lancet 351:490-3.
2. Visapaa I, Fellman V, Vesa J, Dasvarma A, Hutton J, Kumar V, Payne GS, Makarow M, Coster RV, Taylor RW, Turnbull DM, Suomalainen A, Peltonen L (2002) GRACILE syndrome, a lethal metabolic disorder with iron overload, is caused by a point mutation in BCS1L. Am J Hum Genet 71:863-76.
3. Levéen P, Kotarsky H, Mörgelin M, Karikoski R, Elmér E, Fellman V (2011) The GRACILE mutation introduced into Bcs1l causes postnatal complex III deficiency: a viable mouse model for mitochondrial hepatopathy. Hepatology 53:437-47.