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Nerve cell lesions caused by 3-hydroxyglutaric acid: a possible mechanism for neurodegeneration in glutaric acidaemia I.

作者信息

Flott-Rahmel B, Falter C, Schluff P, Fingerhut R, Christensen E, Jakobs C, Musshoff U, Fautek J D, Deufel T, Ludolph A, Ullrich K

机构信息

Department of Pediatrics, University of Münster, Germany.

出版信息

J Inherit Metab Dis. 1997 Jul;20(3):387-90. doi: 10.1023/a:1005342331229.

DOI:10.1023/a:1005342331229
PMID:9266362
Abstract
摘要

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2
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D-2-hydroxyglutaric acidaemia: identification of a new enzyme, D-2-hydroxyglutarate dehydrogenase, localized in mitochondria.D-2-羟基戊二酸血症：一种定位于线粒体的新酶——D-2-羟基戊二酸脱氢酶的鉴定。
J Inherit Metab Dis. 1995;18(2):194-6. doi: 10.1007/BF00711764.
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Impairment of astrocytic glutaminolysis in glutaric aciduria type I.I 型戊二酸血症中天冬氨酸酰谷氨酸合成酶缺陷导致星形胶质细胞谷氨酰胺分解代谢障碍。
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Sensitivity and specificity of free and total glutaric acid and 3-hydroxyglutaric acid measurements by stable-isotope dilution assays for the diagnosis of glutaric aciduria type I.通过稳定同位素稀释分析法测定游离和总戊二酸以及3-羟基戊二酸用于诊断I型戊二酸血症的敏感性和特异性。
J Inherit Metab Dis. 1999 Dec;22(8):867-81. doi: 10.1023/a:1005683222187.

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Int J Neonatal Screen. 2021 Jun 18;7(2):32. doi: 10.3390/ijns7020032.
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本文引用的文献

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Effects of the epileptogenic agent bicuculline methiodide on membrane currents induced by N-methyl-D-aspartate and kainate (oocyte; Xenopus laevis).
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Organotypic monolayer cultures of nervous tissue.神经组织的器官型单层培养物。
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Improved assay of glutaryl-CoA dehydrogenase in cultured cells and liver: application to glutaric aciduria type I.培养细胞和肝脏中戊二酰辅酶A脱氢酶检测方法的改进：应用于I型戊二酸尿症
gcdh-/-小鼠大脑皮层和纹状体中谷氨酸受体和转运体表达增加：对I型戊二酸血症神经病理学的可能影响。
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Neural transplants in patients with Huntington's disease undergo disease-like neuronal degeneration.亨廷顿舞蹈症患者体内的神经移植会经历类似疾病的神经元退化。
Proc Natl Acad Sci U S A. 2009 Jul 28;106(30):12483-8. doi: 10.1073/pnas.0904239106. Epub 2009 Jul 20.
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Biochemistry and bioenergetics of glutaryl-CoA dehydrogenase deficiency.戊二酰辅酶A脱氢酶缺乏症的生物化学与生物能量学
J Inherit Metab Dis. 2007 Oct;30(5):673-80. doi: 10.1007/s10545-007-0678-8. Epub 2007 Sep 21.
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Age and brain structural related effects of glutaric and 3-hydroxyglutaric acids on glutamate binding to plasma membranes during rat brain development.戊二酸和3-羟基戊二酸在大鼠脑发育过程中对谷氨酸与质膜结合的年龄及脑结构相关影响。
Cell Mol Neurobiol. 2007 Sep;27(6):805-18. doi: 10.1007/s10571-007-9197-2. Epub 2007 Sep 5.
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Energy metabolism is compromised in skeletal muscle of rats chronically-treated with glutaric acid.长期用戊二酸处理的大鼠骨骼肌中的能量代谢受到损害。
Metab Brain Dis. 2007 Mar;22(1):111-23. doi: 10.1007/s11011-006-9043-0. Epub 2007 Jan 13.
8
Infant mice with glutaric acidaemia type I have increased vulnerability to 3-nitropropionic acid toxicity.患有I型戊二酸血症的幼鼠对3-硝基丙酸毒性的易感性增加。
J Inherit Metab Dis. 2006 Oct;29(5):612-9. doi: 10.1007/s10545-006-0102-9. Epub 2006 Aug 30.
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Glutaric acid administration impairs energy metabolism in midbrain and skeletal muscle of young rats.
Neurochem Res. 2005 Sep;30(9):1123-31. doi: 10.1007/s11064-005-7711-9.
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Promotion of oxidative stress by 3-hydroxyglutaric acid in rat striatum.
J Inherit Metab Dis. 2005;28(1):57-67. doi: 10.1007/s10545-005-3677-7.
Clin Chim Acta. 1983 Mar 28;129(1):91-7. doi: 10.1016/0009-8981(83)90155-9.
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Amino acids as central nervous transmitters: the influence of ions, amino acid analogues, and ontogeny on transport systems for L-glutamic and L-aspartic acids and glycine into central nervous synaptosomes of the rat.氨基酸作为中枢神经递质：离子、氨基酸类似物及个体发育对大鼠中枢神经突触体摄取L-谷氨酸、L-天冬氨酸及甘氨酸转运系统的影响。
J Neurochem. 1973 Dec;21(6):1533-50. doi: 10.1111/j.1471-4159.1973.tb06037.x.
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Hypothesis: a role for quinolinic acid in the neuropathology of glutaric aciduria type I.
Can J Neurol Sci. 1987 Aug;14(3 Suppl):441-3. doi: 10.1017/s0317167100037872.
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Postnatal development of GABA-mediated synaptic inhibition in rat hippocampus.大鼠海马中γ-氨基丁酸（GABA）介导的突触抑制的产后发育
Neuroscience. 1989;28(3):551-61. doi: 10.1016/0306-4522(89)90004-3.
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Direct observation of the agonist-specific regional vulnerability to glutamate, NMDA, and kainate neurotoxicity in organotypic hippocampal cultures.
Exp Neurol. 1991 Oct;114(1):11-22. doi: 10.1016/0014-4886(91)90079-r.
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Glutaric aciduria type 1 an atypical presentation together with some observations upon treatment and the possible cause of cerebral damage.
Neuropediatrics. 1992 Dec;23(6):322-6. doi: 10.1055/s-2008-1071366.
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Inhibition of brain glutamate decarboxylase by glutarate, glutaconate, and beta-hydroxyglutarate: explanation of the symptoms in glutaric aciduria?
Clin Chim Acta. 1976 Feb 2;66(3):411-5. doi: 10.1016/0009-8981(76)90241-2.
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Glutaric aciduria: biochemical and morphologic considerations.戊二酸尿症：生化及形态学方面的考量
J Pediatr. 1977 May;90(5):746-50. doi: 10.1016/s0022-3476(77)81240-7.