Glutaric aciduria I: Creatine supplementation restores creatinephosphate levels in mixed cortex cells from rat incubated with 3-hydroxyglutarate

Molecular Genetics and Metabolism

Volumn 78, Issue 2, 2003, Pages 108-111

  Das, A M ^a   Lucke T ^a   Ullrich, K ^b  

^a HANNOVER MEDICAL SCHOOL   (Germany)

^b UNIVERSITY OF HAMBURG   (Germany)

Author keywords

ATP; Creatine; Creatinephosphate; Excitotoxicity; Glutaric aciduria; Hydroxyglutarate; Mitochondria; Neurons; Respiratory chain

Indexed keywords

3 HYDROXYGLUTARATE; ADENOSINE DIPHOSPHATE; ADENOSINE PHOSPHATE; ADENOSINE TRIPHOSPHATE; CREATINE; CREATINE PHOSPHATE; DIZOCILPINE; GLUTARIC ACID DERIVATIVE; N METHYL DEXTRO ASPARTIC ACID RECEPTOR; N METHYL DEXTRO ASPARTIC ACID RECEPTOR BLOCKING AGENT; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BLOOD LEVEL; BRAIN CELL; BRAIN CORTEX; BRAIN MITOCHONDRION; CONTROLLED STUDY; ENZYME DEFICIENCY; GLUTARIC ACIDURIA 1; HIGH ENERGY PHOSPHATE; INCUBATION TIME; MEMBRANE DEPOLARIZATION; NEUROLOGIC DISEASE; NEUROPATHOLOGY; NONHUMAN; PHOSPHATE BLOOD LEVEL; PRIORITY JOURNAL; RAT;

EID: 0345701486     PISSN: 10967192     EISSN: None     Source Type: Journal    
DOI: 10.1016/S1096-7192(02)00227-5     Document Type: Article

References (17)

1. Ullrich K., Flott-Rahmel B., Schluff P., Musshoff U., Das A., Lücke T., Steinfeld R., Christensen E., Jakobs C., Ludolph A., Neu A., Röper R. Glutaric aciduria type I: pathomechanisms of neurodegeneration. J. Inher. Metab. Dis. 22:1999;392-403.
2. Gregersen N., Brandt N.J. Ketotic episodes in glutaryl-CoA dehydrogenase deficiency (glutaric aciduria). Pediatr. Res. 13:1979;977-981.
3. Ikonomidou C., Turski L. Excitotoxicity and neurodegenerative diseases. Curr. Opin. Neurol. 8:1995;487-497.
4. Goslin K., Banker G. Experimental observations on the development of polarity by hippocampal neurons in culture. J. Cell Biol. 108:1989;1507-1516.
5. Ouhabi R., Boue-Grabot M., Mazat J. ATP synthesis in permeabilized cells: assessment of the ATP/O ratios in situ. Mod. Trends Bio Thermo Kinet. 3:1994;141-144.
6. Ronner P., Friel E., Czerniawski K., Frankle S. Luminometric assay of ATP, phosphocreatine, and creatine for estimation of free ADP and free AMP. Anal. Biochem. 275:1999;208-216.
7. Harris D.A. Spectrophotometric assays. Harris D.A., Bashford C.L. Spectrophotometry and Spectrofluorimetry. 1987;74-77 IRL Press, Oxford.
8. Bensadoun A., Weinstein D. Assay of proteins in the presence of interfering material. Anal. Biochem. 70:1976;241-250.
9. Lundin A., Richardson A., Thore A. Continuous monitoring of ATP-converting reactions by purified firefly luciferase. Anal. Biochem. 75:1976;611-620.
10. Koh J.Y., Choi D.W. Quantitative determination of glutamate mediated cortical neuronal injury in cell culture by lactate dehydrogenase efflux assay. J. Neurosci. Meth. 20:1987;83-90.
11. Fitch C.D., Shields R.P., Payne W.F., Dacus J.M. Creatine metabolism in skeletal muscle. Specificity of the creatine entry process. J. Biol. Chem. 243:1968;2024-2027.
12. Möller A., Hamprecht B. Creatine transport in cultured cells of rat and mouse brain. J. Neurochem. 52:1989;544-550.
13. Guerrero-Ontiveros M.L., Wallimann T. Creatine supplementation in health and disease. Effects of chronic creatine ingestion in vivo: down-regulation of the expression of creatine transporter isoforms in skeletal muscle. Mol. Cell. Biochem. 184:1998;427-437.
14. Gulmbal C., Kilimann M.W. A Na-dependent creatine transporter in rabbit brain, muscle, heart and kidney. J. Biol. Chem. 268:1993;8418-8421.
15. Stachowiak O., Schlattner U., Dolder M., Wallimann T. Oligomeric state and membrane binding behaviour of creatine kinase isoenzymes: implications for cellular function and mitochondrial structure. Mol. Cell. Biochem. 184:1998;141-151.
16. Müller M., Cheneval D., Carafoli E. The mitochondrial creatine phosphokinase is associated with inner membrane cardiolipin. Brautbar N. Myocardial and Skeletal Muscle Bioenergetics. 1985;51-155 Plenum, New York.
17. Kölker S., Ahlemeyer B., Krieglstein J., Hoffmann G.F. Contribution of reactive oxygen species to 3-hydroxyglutarate neurotoxicity in primary neuronal cultures from chick embryo telencephalons. Pediatr. Res. 50:2001;76-82.