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REVIEW: Practical strategies to maintain anabolism by intravenous nutritional management in children with inborn metabolic diseases.
Kripps KA, Baker PR 2nd, Thomas JA, Skillman HE, Bernstein L, Gaughan S, Burns C, Coughlin CR 2nd, McCandless SE, Larson AA, Kochar A, Stillman CF, Wymore EM, Hendricks EG, Woontner M, Van Hove JLK. Kripps KA, et al. Among authors: woontner m. Mol Genet Metab. 2021 Jul;133(3):231-241. doi: 10.1016/j.ymgme.2021.04.007. Epub 2021 May 7. Mol Genet Metab. 2021. PMID: 33985889 Free article. Review.
Increased glutamate receptor and transporter expression in the cerebral cortex and striatum of gcdh-/- mice: possible implications for the neuropathology of glutaric acidemia type I.
Lagranha VL, Matte U, de Carvalho TG, Seminotti B, Pereira CC, Koeller DM, Woontner M, Goodman SI, de Souza DO, Wajner M. Lagranha VL, et al. Among authors: woontner m. PLoS One. 2014 Mar 4;9(3):e90477. doi: 10.1371/journal.pone.0090477. eCollection 2014. PLoS One. 2014. PMID: 24594605 Free PMC article.
Experimental evidence that bioenergetics disruption is not mainly involved in the brain injury of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload.
Amaral AU, Cecatto C, Seminotti B, Ribeiro CA, Lagranha VL, Pereira CC, de Oliveira FH, de Souza DG, Goodman S, Woontner M, Wajner M. Amaral AU, et al. Among authors: woontner m. Brain Res. 2015 Sep 16;1620:116-29. doi: 10.1016/j.brainres.2015.05.013. Epub 2015 May 18. Brain Res. 2015. PMID: 25998543
Higher Vulnerability of Menadione-Exposed Cortical Astrocytes of Glutaryl-CoA Dehydrogenase Deficient Mice to Oxidative Stress, Mitochondrial Dysfunction, and Cell Death: Implications for the Neurodegeneration in Glutaric Aciduria Type I.
Rodrigues MDN, Seminotti B, Zanatta Â, de Mello Gonçalves A, Bellaver B, Amaral AU, Quincozes-Santos A, Goodman SI, Woontner M, Souza DO, Wajner M. Rodrigues MDN, et al. Among authors: woontner m. Mol Neurobiol. 2017 Aug;54(6):4795-4805. doi: 10.1007/s12035-016-0023-z. Epub 2016 Aug 10. Mol Neurobiol. 2017. PMID: 27510504
Management of a patient with holocarboxylase synthetase deficiency.
Van Hove JL, Josefsberg S, Freehauf C, Thomas JA, Thuy le P, Barshop BA, Woontner M, Mock DM, Chiang PW, Spector E, Meneses-Morales I, Cervantes-Roldán R, León-Del-Río A. Van Hove JL, et al. Among authors: woontner m. Mol Genet Metab. 2008 Dec;95(4):201-5. doi: 10.1016/j.ymgme.2008.09.006. Epub 2008 Oct 29. Mol Genet Metab. 2008. PMID: 18974016 Free PMC article.
[No title available]
[No authors listed] [No authors listed] PMID: 33354834
The M405V allele of the glutaryl-CoA dehydrogenase gene is an important marker for glutaric aciduria type I (GA-I) low excretors.
Schillaci LA, Greene CL, Strovel E, Rispoli-Joines J, Spector E, Woontner M, Scharer G, Enns GM, Gallagher R, Zinn AB, McCandless SE, Hoppel CL, Goodman SI, Bedoyan JK. Schillaci LA, et al. Among authors: woontner m. Mol Genet Metab. 2016 Sep;119(1-2):50-6. doi: 10.1016/j.ymgme.2016.06.012. Epub 2016 Jul 1. Mol Genet Metab. 2016. PMID: 27397597
38 results