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Transport and distribution of 3-hydroxyglutaric acid before and during induced encephalopathic crises in a mouse model of glutaric aciduria type 1

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Transport and distribution of 3-hydroxyglutaric acid before and during induced encephalopathic crises in a mouse model of glutaric aciduria type 1. / Keyser, Britta; Glatzel, Markus; Stellmer, Franziska; Kortmann, Bastian; Lukacs, Zoltan; Kölker, Stefan; Sauer, Sven W; Muschol, Nicole; Herdering, Wilhelm; Thiem, Joachim; Goodman, Stephen I; Koeller, David M; Ullrich, Kurt; Braulke, Thomas; Mühlhausen, Chris.

In: BBA-MOL BASIS DIS, Vol. 1782, No. 6, 6, 06.2008, p. 385-390.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Keyser, B, Glatzel, M, Stellmer, F, Kortmann, B, Lukacs, Z, Kölker, S, Sauer, SW, Muschol, N, Herdering, W, Thiem, J, Goodman, SI, Koeller, DM, Ullrich, K, Braulke, T & Mühlhausen, C 2008, 'Transport and distribution of 3-hydroxyglutaric acid before and during induced encephalopathic crises in a mouse model of glutaric aciduria type 1', BBA-MOL BASIS DIS, vol. 1782, no. 6, 6, pp. 385-390. https://doi.org/10.1016/j.bbadis.2008.02.008

APA

Keyser, B., Glatzel, M., Stellmer, F., Kortmann, B., Lukacs, Z., Kölker, S., Sauer, S. W., Muschol, N., Herdering, W., Thiem, J., Goodman, S. I., Koeller, D. M., Ullrich, K., Braulke, T., & Mühlhausen, C. (2008). Transport and distribution of 3-hydroxyglutaric acid before and during induced encephalopathic crises in a mouse model of glutaric aciduria type 1. BBA-MOL BASIS DIS, 1782(6), 385-390. [6]. https://doi.org/10.1016/j.bbadis.2008.02.008

Vancouver

Keyser B, Glatzel M, Stellmer F, Kortmann B, Lukacs Z, Kölker S et al. Transport and distribution of 3-hydroxyglutaric acid before and during induced encephalopathic crises in a mouse model of glutaric aciduria type 1. BBA-MOL BASIS DIS. 2008 Jun;1782(6):385-390. 6. https://doi.org/10.1016/j.bbadis.2008.02.008

Bibtex

@article{687a6671577b46dba76a981a7eb7eee1,
title = "Transport and distribution of 3-hydroxyglutaric acid before and during induced encephalopathic crises in a mouse model of glutaric aciduria type 1",
abstract = "Glutaric aciduria type 1 (GA1) is caused by the deficiency of glutaryl-CoA dehydrogenase (GCDH). Affected patients are prone to the development of encephalopathic crises during an early time window with destruction of striatal neurons and a subsequent irreversible movement disorder. 3-Hydroxyglutaric acid (3OHGA) accumulates in tissues and body fluids of GA1 patients and has been shown to mediate toxic effects on neuronal as well as endothelial cells. Injection of (3H)-labeled into 6 week-old Gcdh(-/-) mice, a model of GA1, revealed a low recovery in kidney, liver, or brain tissue that did not differ from control mice. Significant amounts of 3OHGA were found to be excreted via the intestinal tract. Exposure of Gcdh(-/-) mice to a high protein diet led to an encephalopathic crisis, vacuolization in the brain, and death after 4-5 days. Under these conditions, high amounts of injected 3H-3OHGA were found in kidneys of Gcdh(-/-) mice, whereas the radioactivity recovered in brain and blood was reduced. The data demonstrate that under conditions mimicking encephalopathic crises the blood-brain barrier appears to remain intact.",
keywords = "Amino Acid Metabolism, Inborn Errors/complications, Animals, Biological Transport, Brain/metabolism, Brain Diseases/etiology, Dextrans/metabolism, Disease Models, Animal, Glutarates/metabolism, Glutaryl-CoA Dehydrogenase/deficiency, Humans, Immunohistochemistry, Intestinal Mucosa/metabolism, Kidney/metabolism, Mice, Mice, Inbred C57BL, Mice, Knockout",
author = "Britta Keyser and Markus Glatzel and Franziska Stellmer and Bastian Kortmann and Zoltan Lukacs and Stefan K{\"o}lker and Sauer, {Sven W} and Nicole Muschol and Wilhelm Herdering and Joachim Thiem and Goodman, {Stephen I} and Koeller, {David M} and Kurt Ullrich and Thomas Braulke and Chris M{\"u}hlhausen",
year = "2008",
month = jun,
doi = "10.1016/j.bbadis.2008.02.008",
language = "English",
volume = "1782",
pages = "385--390",
journal = "BBA-MOL BASIS DIS",
issn = "0925-4439",
publisher = "Elsevier",
number = "6",

}

RIS

TY - JOUR

T1 - Transport and distribution of 3-hydroxyglutaric acid before and during induced encephalopathic crises in a mouse model of glutaric aciduria type 1

AU - Keyser, Britta

AU - Glatzel, Markus

AU - Stellmer, Franziska

AU - Kortmann, Bastian

AU - Lukacs, Zoltan

AU - Kölker, Stefan

AU - Sauer, Sven W

AU - Muschol, Nicole

AU - Herdering, Wilhelm

AU - Thiem, Joachim

AU - Goodman, Stephen I

AU - Koeller, David M

AU - Ullrich, Kurt

AU - Braulke, Thomas

AU - Mühlhausen, Chris

PY - 2008/6

Y1 - 2008/6

N2 - Glutaric aciduria type 1 (GA1) is caused by the deficiency of glutaryl-CoA dehydrogenase (GCDH). Affected patients are prone to the development of encephalopathic crises during an early time window with destruction of striatal neurons and a subsequent irreversible movement disorder. 3-Hydroxyglutaric acid (3OHGA) accumulates in tissues and body fluids of GA1 patients and has been shown to mediate toxic effects on neuronal as well as endothelial cells. Injection of (3H)-labeled into 6 week-old Gcdh(-/-) mice, a model of GA1, revealed a low recovery in kidney, liver, or brain tissue that did not differ from control mice. Significant amounts of 3OHGA were found to be excreted via the intestinal tract. Exposure of Gcdh(-/-) mice to a high protein diet led to an encephalopathic crisis, vacuolization in the brain, and death after 4-5 days. Under these conditions, high amounts of injected 3H-3OHGA were found in kidneys of Gcdh(-/-) mice, whereas the radioactivity recovered in brain and blood was reduced. The data demonstrate that under conditions mimicking encephalopathic crises the blood-brain barrier appears to remain intact.

AB - Glutaric aciduria type 1 (GA1) is caused by the deficiency of glutaryl-CoA dehydrogenase (GCDH). Affected patients are prone to the development of encephalopathic crises during an early time window with destruction of striatal neurons and a subsequent irreversible movement disorder. 3-Hydroxyglutaric acid (3OHGA) accumulates in tissues and body fluids of GA1 patients and has been shown to mediate toxic effects on neuronal as well as endothelial cells. Injection of (3H)-labeled into 6 week-old Gcdh(-/-) mice, a model of GA1, revealed a low recovery in kidney, liver, or brain tissue that did not differ from control mice. Significant amounts of 3OHGA were found to be excreted via the intestinal tract. Exposure of Gcdh(-/-) mice to a high protein diet led to an encephalopathic crisis, vacuolization in the brain, and death after 4-5 days. Under these conditions, high amounts of injected 3H-3OHGA were found in kidneys of Gcdh(-/-) mice, whereas the radioactivity recovered in brain and blood was reduced. The data demonstrate that under conditions mimicking encephalopathic crises the blood-brain barrier appears to remain intact.

KW - Amino Acid Metabolism, Inborn Errors/complications

KW - Animals

KW - Biological Transport

KW - Brain/metabolism

KW - Brain Diseases/etiology

KW - Dextrans/metabolism

KW - Disease Models, Animal

KW - Glutarates/metabolism

KW - Glutaryl-CoA Dehydrogenase/deficiency

KW - Humans

KW - Immunohistochemistry

KW - Intestinal Mucosa/metabolism

KW - Kidney/metabolism

KW - Mice

KW - Mice, Inbred C57BL

KW - Mice, Knockout

U2 - 10.1016/j.bbadis.2008.02.008

DO - 10.1016/j.bbadis.2008.02.008

M3 - SCORING: Journal article

C2 - 18348873

VL - 1782

SP - 385

EP - 390

JO - BBA-MOL BASIS DIS

JF - BBA-MOL BASIS DIS

SN - 0925-4439

IS - 6

M1 - 6

ER -