Understanding cerebral L-lysine metabolism: the role of L-pipecolate metabolism in Gcdh-deficient mice as a model for glutaric aciduria type I

Autor: Silvana Opp, Georg F. Hoffmann, Roland Posset, Stefan Kölker, Jürgen G. Okun, Sven W. Sauer, Alfred Völkl, Eduard A. Struys, Heribert Mohr

Publikováno v: Journal of Inherited Metabolic Disease, 38(2), 265-272. Springer Netherlands
Posset, R, Opp, S, Struys, E A, Volkl, A, Mohr, H, Hoffmann, G F, Kolker, S, Sauer, S W & Okun, J G 2015, ' Understanding cerebral L-lysine metabolism: the role of L-pipecolate metabolism in Gcdh-deficient mice as a model for glutaric aciduria type I ', Journal of Inherited Metabolic Disease, vol. 38, no. 2, pp. 265-272 . https://doi.org/10.1007/s10545-014-9762-z

Inherited deficiencies of the L-lysine catabolic pathway cause glutaric aciduria type I and pyridoxine-dependent epilepsy. Dietary modulation of cerebral L-lysine metabolism is thought to be an important therapeutic intervention for these diseases. T

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2751f5e2f5e31c4240ced916a6200fd3
https://doi.org/10.1007/s10545-014-9762-z

Isolation of rat hepatic peroxisomes by means of immune free flow electrophoresis

Autor: H. Dariush Fahimi, Alfred Völkl, Heribert Mohr, Gerhard Weber

Publikováno v: Electrophoresis. 18:774-780

Rat hepatic peroxisomes (PO) were separated from other cell organelles by free flow electrophoresis (FFE) in combination with immunocomplexing PO prior to FFE with an antibody directed against the cytoplasmic aspect of the peroxisomal membrane protei

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::97026b2be953a5bdcad77dbdc844093f
https://doi.org/10.1002/elps.1150180519

Peroxisome subpopulations of the rat liver. Isolation by immune free flow electrophoresis

Autor: Heribert Mohr, H D Fahimi, Alfred Völkl

Publikováno v: The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society. 47(9)

Peroxisomes (POs) are a heterogenous population of cell organelles which, in mammals, are most abundant in liver and kidney. Although they are usually isolated by differential and density gradient centrifugation, isolation is hampered by their high f

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e05d0093c3b9f7169e9d5c39850c8138
https://pubmed.ncbi.nlm.nih.gov/10449531

Immuno-isolation of highly purified peroxisomes using magnetic beads and continuous immunomagnetic sorting

Autor: Georg H. Lüers, Roland Hartig, H D Fahimi, Michael Hausmann, Christoph Prof Dr Dr Cremer, Alfred Völkl, Heribert Mohr

Publikováno v: Electrophoresis. 19(7)

Immuno-isolation is a powerful technique for the isolation of cells as well as subcellular organelle populations based on their antigenic properties. We have established a method for immuno-isolation of peroxisomes (PO) from both rat liver and the hu

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::df122456abd5bfb9aeb6572d73be25cb
https://pubmed.ncbi.nlm.nih.gov/9662184