Literature DB >> 18459129

Disturbed hepatic carbohydrate management during high metabolic demand in medium-chain acyl-CoA dehydrogenase (MCAD)-deficient mice.

Hilde Herrema1, Terry G J Derks, Theo H van Dijk, Vincent W Bloks, Albert Gerding, Rick Havinga, Uwe J F Tietge, Michael Müller, G Peter A Smit, Folkert Kuipers, Dirk-Jan Reijngoud.   

Abstract

UNLABELLED: Medium-chain acyl-coenzyme A (CoA) dehydrogenase (MCAD) catalyzes crucial steps in mitochondrial fatty acid oxidation, a process that is of key relevance for maintenance of energy homeostasis, especially during high metabolic demand. To gain insight into the metabolic consequences of MCAD deficiency under these conditions, we compared hepatic carbohydrate metabolism in vivo in wild-type and MCAD(-/-) mice during fasting and during a lipopolysaccharide (LPS)-induced acute phase response (APR). MCAD(-/-) mice did not become more hypoglycemic on fasting or during the APR than wild-type mice did. Nevertheless, microarray analyses revealed increased hepatic peroxisome proliferator-activated receptor gamma coactivator-1alpha (Pgc-1alpha) and decreased peroxisome proliferator-activated receptor alpha (Ppar alpha) and pyruvate dehydrogenase kinase 4 (Pdk4) expression in MCAD(-/-) mice in both conditions, suggesting altered control of hepatic glucose metabolism. Quantitative flux measurements revealed that the de novo synthesis of glucose-6-phosphate (G6P) was not affected on fasting in MCAD(-/-) mice. During the APR, however, this flux was significantly decreased (-20%) in MCAD(-/-) mice compared with wild-type mice. Remarkably, newly formed G6P was preferentially directed toward glycogen in MCAD(-/-) mice under both conditions. Together with diminished de novo synthesis of G6P, this led to a decreased hepatic glucose output during the APR in MCAD(-/-) mice; de novo synthesis of G6P and hepatic glucose output were maintained in wild-type mice under both conditions. APR-associated hypoglycemia, which was observed in wild-type mice as well as MCAD(-/-) mice, was mainly due to enhanced peripheral glucose uptake.
CONCLUSION: Our data demonstrate that MCAD deficiency in mice leads to specific changes in hepatic carbohydrate management on exposure to metabolic stress. This deficiency, however, does not lead to reduced de novo synthesis of G6P during fasting alone, which may be due to the existence of compensatory mechanisms or limited rate control of MCAD in murine mitochondrial fatty acid oxidation.

Entities:  

Mesh:

Substances:

Year:  2008        PMID: 18459129     DOI: 10.1002/hep.22284

Source DB:  PubMed          Journal:  Hepatology        ISSN: 0270-9139            Impact factor:   17.425


  18 in total

Review 1.  Mitochondrial fatty acid oxidation disorders: pathophysiological studies in mouse models.

Authors:  Ute Spiekerkoetter; Philip A Wood
Journal:  J Inherit Metab Dis       Date:  2010-06-08       Impact factor: 4.982

Review 2.  Systems biology from micro-organisms to human metabolic diseases: the role of detailed kinetic models.

Authors:  Barbara M Bakker; Karen van Eunen; Jeroen A L Jeneson; Natal A W van Riel; Frank J Bruggeman; Bas Teusink
Journal:  Biochem Soc Trans       Date:  2010-10       Impact factor: 5.407

3.  Clinical and biological features at diagnosis in mitochondrial fatty acid beta-oxidation defects: a French pediatric study of 187 patients.

Authors:  Julien Baruteau; Philippe Sachs; Pierre Broué; Michèle Brivet; Hendy Abdoul; Christine Vianey-Saban; Hélène Ogier de Baulny
Journal:  J Inherit Metab Dis       Date:  2012-10-03       Impact factor: 4.982

Review 4.  Current issues regarding treatment of mitochondrial fatty acid oxidation disorders.

Authors:  Ute Spiekerkoetter; Jean Bastin; Melanie Gillingham; Andrew Morris; Frits Wijburg; Bridget Wilcken
Journal:  J Inherit Metab Dis       Date:  2010-09-10       Impact factor: 4.982

5.  Medium-chain acyl-Coenzyme A dehydrogenase deficiency (MCADD): a cause of severe hypoglycaemia in an apparently well child.

Authors:  Kene Ebuka Maduemem
Journal:  BMJ Case Rep       Date:  2016-11-30

Review 6.  Regulation of pyruvate metabolism in metabolic-related diseases.

Authors:  Nam Ho Jeoung; Chris R Harris; Robert A Harris
Journal:  Rev Endocr Metab Disord       Date:  2014-03       Impact factor: 6.514

7.  SIRT3 regulates mitochondrial fatty-acid oxidation by reversible enzyme deacetylation.

Authors:  Matthew D Hirschey; Tadahiro Shimazu; Eric Goetzman; Enxuan Jing; Bjoern Schwer; David B Lombard; Carrie A Grueter; Charles Harris; Sudha Biddinger; Olga R Ilkayeva; Robert D Stevens; Yu Li; Asish K Saha; Neil B Ruderman; James R Bain; Christopher B Newgard; Robert V Farese; Frederick W Alt; C Ronald Kahn; Eric Verdin
Journal:  Nature       Date:  2010-03-04       Impact factor: 49.962

8.  Unacylated ghrelin rapidly modulates lipogenic and insulin signaling pathway gene expression in metabolically active tissues of GHSR deleted mice.

Authors:  Patric J D Delhanty; Yuxiang Sun; Jenny A Visser; Anke van Kerkwijk; Martin Huisman; Wilfred F J van Ijcken; Sigrid Swagemakers; Roy G Smith; Axel P N Themmen; Aart-Jan van der Lely
Journal:  PLoS One       Date:  2010-07-26       Impact factor: 3.240

9.  Does LKB1 mediate activation of hepatic AMP-protein kinase (AMPK) and sirtuin1 (SIRT1) after Roux-en-Y gastric bypass in obese rats?

Authors:  Yanhua Peng; Drew A Rideout; Steven S Rakita; William R Gower; Min You; Michel M Murr
Journal:  J Gastrointest Surg       Date:  2010-02       Impact factor: 3.452

10.  Experimental evidence for protein oxidative damage and altered antioxidant defense in patients with medium-chain acyl-CoA dehydrogenase deficiency.

Authors:  Terry G J Derks; Catharina M L Touw; Graziela S Ribas; Giovana B Biancini; Camila S Vanzin; Giovanna Negretto; Caroline P Mescka; Dirk Jan Reijngoud; G Peter A Smit; Moacir Wajner; Carmen R Vargas
Journal:  J Inherit Metab Dis       Date:  2014-03-13       Impact factor: 4.982

View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.