caa a22 4500 467933847 CHVBK 20180406152951.0 cr unu---uuuuu 170328e20061001xx s 000 0 eng 10.1007/s10545-006-0102-9 doi (NATIONALLICENCE)springer-10.1007/s10545-006-0102-9 Infant mice with glutaric acidaemia type I have increased vulnerability to 3-nitropropionic acid toxicity [Elektronische Daten] [K. Bjugstad, L. Crnic, S. Goodman, C. Freed] Summary: Glutaric acidaemia type I (GA I) is an inborn error of metabolism caused by a deficiency of glutaryl-CoA dehydrogenase (GCDH) and is characterized clinically by striatal degeneration that almost always occurs in early childhood. A murine knockout model of GA I has the organic aciduria seen in the human disorder, but this model does not develop striatal degeneration spontaneously. 3-Nitropropionic acid (3NP), a succinic dehydrogenase inhibitor with specificity for the striatum, was investigated as a potential initiator of striatal degeneration in GCDH-deficient mice. This study shows that GCDH-deficient mouse pups are more susceptible to 3NP than their wild-type littermates, and that all mouse pups are more sensitive to 3NP as infants than as adolescents and adults. Increased sensitivity to 3NP early in life may model the developmental window for the striatal damage observed in human GA I. SSIEM and Springer, 2006 Bjugstad K. Department Psychiatry, University of Colorado Health Sciences Center, Denver, Colorado, USA aut Crnic L. Department Pediatrics, University of Colorado Health Sciences Center, Denver, Colorado, USA aut Goodman S. Department Pediatrics, University of Colorado Health Sciences Center, Denver, Colorado, USA aut Freed C. Department Medicine and Division of Clinical Pharmacology, University of Colorado Health Sciences Center, Denver, Colorado, USA aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/5(2006-10-01), 612-619 0141-8955 29:5<612 2006 29 10545 https://doi.org/10.1007/s10545-006-0102-9 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10545-006-0102-9 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Bjugstad K. Department Psychiatry, University of Colorado Health Sciences Center, Denver, Colorado, USA aut NATIONALLICENCE 700 1- Crnic L. Department Pediatrics, University of Colorado Health Sciences Center, Denver, Colorado, USA aut NATIONALLICENCE 700 1- Goodman S. Department Pediatrics, University of Colorado Health Sciences Center, Denver, Colorado, USA aut NATIONALLICENCE 700 1- Freed C. Department Medicine and Division of Clinical Pharmacology, University of Colorado Health Sciences Center, Denver, Colorado, USA aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/5(2006-10-01), 612-619 0141-8955 29:5<612 2006 29 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer