Our research is aimed at a full understanding of carbohydrate metabolism in the liver and other animal tissues; in a parallel and complementary way we tend to give a biochemical explanation to the related congenital disorders allowing eventually an appropriate treatment. During the next few years, our research will be more specifically oriented to an investigation of the role of fructose 2,6-bisphosphate in the control of metabolism in various tissues and to a characterization of the proteins bound to particulate glycogen in the liver; we will also try to detect inborn errors of metabolism related to the formation or degradation of fructose 2,6-bisphosphate. Diseases of lysosomes and peroxisomes will also be studied, in parallel with basic research on these organelles. Liver and muscle biopsies from patients affected by various inborn errors of metabolism (mostly glycogen storage disease, fructose intolerance, congenital lactic acidosis, galactosemia, inborn lysosomal diseases) are regularly received in this laboratory from many clinical centers located mostly in western Europe but also in South America and other countries. These biopsies will be submitted to both biochemical and ultrastructural investigation. Experimental work on fructose 2,6-bisphosphate will be facilitated by an exquisitely sensitive assay for this compound and will involve (1) the purification and a comparative study of phosphofructo 2-kinase and fructose 2,6-bisphosphatase from various origin, also making use of monoclonal antibodies directed against these proteins; (2) the search for new enzymes using fructose 2,6-bisphosphate as a substrate or as a cofactor; (3) a characterization of the protein phosphatases involved in the dephosphorylation of phosphofructo 2-kinase. For the study of glycogen-bound enzymes, particulate glycogen will be isolated by a new extremely rapid procedure.

Agency
National Institute of Health (NIH)
Institute
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Type
Research Project (R01)
Project #
5R01DK009235-22
Application #
3224604
Study Section
Biochemistry Study Section (BIO)
Project Start
1977-06-01
Project End
1989-07-31
Budget Start
1987-08-01
Budget End
1988-07-31
Support Year
22
Fiscal Year
1987
Total Cost
Indirect Cost
Name
Catholic University of Louvain
Department
Type
DUNS #
City
Louvain-LA-Neuve
State
Country
Belgium
Zip Code
Vamecq, J; Draye, J P; Poupaert, J H (1990) Studies on the metabolism of glycolyl-CoA. Biochem Cell Biol 68:846-51
Vamecq, J (1990) Fluorometric assay of peroxisomal oxidases. Anal Biochem 186:340-9
Vamecq, J; Grataroli, R; Draye, J P et al. (1989) Effects of dietary corn oil and salmon oil on the oxidation of fatty acids and prostaglandin E2 in rat gastric mucosa. Prostaglandins 37:335-44
Draye, J P; Vamecq, J (1989) The gluconeogenicity of fatty acids in mammals. Trends Biochem Sci 14:478-9
Vamecq, J; Draye, J P (1989) Comparison between the formation and the oxidation of dicarboxylylcarnitine esters in rat liver and skeletal muscle: possible implications for human inborn disorders of mitochondrial beta-oxidation. J Inherit Metab Dis 12:58-63
Vamecq, J; Draye, J P (1989) Peroxisomal and mitochondrial beta-oxidation of monocarboxylyl-CoA, omega-hydroxymonocarboxylyl-CoA and dicarboxylyl-CoA esters in tissues from untreated and clofibrate-treated rats. J Biochem 106:216-22
Veitch, K; Draye, J P; Van Hoof, F et al. (1988) Effects of riboflavin deficiency and clofibrate treatment on the five acyl-CoA dehydrogenases in rat liver mitochondria. Biochem J 254:477-81
Vamecq, J; Poll-The, B T; Saudubray, J M (1988) [Biochemistry of peroxisome and peroxisomal diseases] Ann Biol Clin (Paris) 46:233-58
Draye, J P; Veitch, K; Vamecq, J et al. (1988) Comparison of the metabolism of dodecanedioic acid in vivo in control, riboflavin-deficient and clofibrate-treated rats. Eur J Biochem 178:183-9
Van Hoof, F; Vamecq, J; Draye, J P et al. (1988) The catabolism of medium- and long-chain dicarboxylic acids. Biochem Soc Trans 16:423-4

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