[PDF] Cerebral haemodynamics in patients with glutaryl-coenzyme A dehydrogenase deficiency. | Semantic Scholar Skip to search formSkip to main content> Semantic Scholar's Logo Search Sign InCreate Free Account You are currently offline. Some features of the site may not work correctly. DOI:10.1093/brain/awp297 Corpus ID: 22599756Cerebral haemodynamics in patients with glutaryl-coenzyme A dehydrogenase deficiency. @article{Strauss2010CerebralHI, title={Cerebral haemodynamics in patients with glutaryl-coenzyme A dehydrogenase deficiency.}, author={K. Strauss and P. Donnelly and M. Wintermark}, journal={Brain : a journal of neurology}, year={2010}, volume={133 Pt 1}, pages={ 76-92 } } K. Strauss, P. Donnelly, M. Wintermark Published 2010 Medicine Brain : a journal of neurology In glutaric aciduria type 1, glutaryl-coenzyme A and its derivatives are produced from intracerebral lysine and entrapped at high concentrations within the brain, where they interfere with energy metabolism. Biochemical toxicity is thought to trigger stroke-like striatal degeneration in susceptible children under 2 years of age. Here, we explore vascular derangements that might also contribute to brain damage. We studied injured and non-injured Amish glutaric aciduria type 1 patients using… Expand View on PubMed academic.oup.com Save to Library Create Alert Cite Launch Research Feed Share This Paper 44 CitationsHighly Influential Citations 3 Background Citations 25 Results Citations 1 View All Figures, Tables, and Topics from this paper table 1 figure 1 figure 2 figure 3 figure 4 figure 5 figure 6 figure 7 figure 8 figure 9 figure 10 figure 11 View All 12 Figures & Tables Coenzymes Necrosis Adverse reaction to drug Glutaric aciduria, type 1 Cerebral Blood Volume Retinal Hemorrhage Cerebrovascular Circulation Brain Injuries Coenzyme A X-Ray Computed Tomography Ultrasonography Glutaryl-CoA dehydrogenase Cerebrovascular accident Structure of middle cerebral artery White matter Structure of basilar artery Gray Matter Homeostasis Blood capillaries Structure of cerebral artery Gastritis, Atrophic Edema Lysine Infant, Newborn acetylsalicylic acid lysinate 44 Citations Citation Type Citation Type All Types Cites Results Cites Methods Cites Background Has PDF Publication Type Author More Filters More Filters Filters Sort by Relevance Sort by Most Influenced Papers Sort by Citation Count Sort by Recency Impairment of astrocytic glutaminolysis in glutaric aciduria type I Shoko Komatsuzaki, Raga Deepthi Ediga, J. 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Morton Medicine Molecular genetics and metabolism 2020 2 View 2 excerpts, cites background Save Alert Research Feed Electroencephalography and transcranial Doppler ultrasonography in neonatal citrullinemia. P. Su, J. Chen, Y. Chen, Dau-Ming Niu, Ju-Hui Hsu, Inn-Chi Lee Medicine Journal of the Formosan Medical Association = Taiwan yi zhi 2014 2 Save Alert Research Feed Current concepts in organic acidurias: understanding intra- and extracerebral disease manifestation S. Kölker, P. Burgard, S. Sauer, J. Okun Biology, Medicine Journal of Inherited Metabolic Disease 2013 41 View 2 excerpts, cites background Save Alert Research Feed ... 1 2 3 4 5 ... References SHOWING 1-10 OF 98 REFERENCES SORT BYRelevance Most Influenced Papers Recency Multimodal imaging of striatal degeneration in Amish patients with glutaryl-CoA dehydrogenase deficiency. K. Strauss, J. Lazovic, M. Wintermark, D. Morton Medicine Brain : a journal of neurology 2007 78 PDF View 29 excerpts, references background and methods Save Alert Research Feed Excitotoxicity and bioenergetics in glutaryl-CoA dehydrogenase deficiency S. Kölker, D. Koeller, +5 authors J. Okun Biology, Medicine Journal of Inherited Metabolic Disease 2004 40 View 2 excerpts, references background Save Alert Research Feed Dynamic changes of striatal and extrastriatal abnormalities in glutaric aciduria type I. I. Harting, E. Neumaier-Probst, +10 authors S. Kölker Psychology, Medicine Brain : a journal of neurology 2009 126 PDF View 3 excerpts, references background Save Alert Research Feed Mechanism of age-dependent susceptibility and novel treatment strategy in glutaric acidemia type I. W. Zinnanti, J. Lazovic, +8 authors K. 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Part C, Seminars in medical genetics 2003 117 View 1 excerpt, references background Save Alert Research Feed Bioenergetics in Glutaryl-Coenzyme A Dehydrogenase Deficiency S. Sauer, J. Okun, +5 authors S. Kölker Biology, Medicine Journal of Biological Chemistry 2005 108 PDF View 3 excerpts, references background Save Alert Research Feed Transport and distribution of 3-hydroxyglutaric acid before and during induced encephalopathic crises in a mouse model of glutaric aciduria type 1. B. Keyser, M. Glatzel, +12 authors C. Mühlhausen Biology, Medicine Biochimica et biophysica acta 2008 24 PDF View 2 excerpts, references background Save Alert Research Feed A diet-induced mouse model for glutaric aciduria type I. W. Zinnanti, J. Lazovic, +6 authors K. Cheng Biology, Medicine Brain : a journal of neurology 2006 87 Highly Influential PDF View 6 excerpts, references background Save Alert Research Feed ... 1 2 3 4 5 ... 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