المؤلفون: Van Beersel, Guillaume, Jadot, Michel, Hamer, Isabelle, Arnould, Thierry

المصدر: Van Beersel , G , Jadot , M , Hamer , I & Arnould , T 2013 , ' Activation of Sterol-Responsive Element Binding Proteins in a Sucrose-Induced Model of Lysosomal Storage ' , The Open Pathology Journal , vol. 7 , 7 , pp. 16-27 . https://doi.org/10.2174/1874375720130905003Test

مصطلحات موضوعية: FASN: fatty acid synthase, HMG-CoA synthase: 3-hydroxy-3-methylglutaryl- Coenzyme A synthase, LDLr: low density lipoprotein receptor, LSDs: lysosomal storage diseases, microtubule-associated protein 1-light chain 3 beta: MAP1-LC3beta/LC3, MVK: mevalonate kinase, SCD-1/2: stearoyl-CoA desaturase 1/2, SREBPs: Sterol Regulatory Element Binding Proteins

وصف الملف: application/pdf

العلاقة: https://researchportal.unamur.be/en/publications/a0fb6eaf-6d2b-41a2-9015-ba6a354f3de3Test

الإتاحة: https://doi.org/10.2174/1874375720130905003Test
https://researchportal.unamur.be/en/publications/a0fb6eaf-6d2b-41a2-9015-ba6a354f3de3Test
https://pure.unamur.be/ws/files/11780011/Van_Beersel_et_al._The_Open_Pathology_Journal_2013.pdfTest
http://www.benthamscience.com/open/topatj/articles/V007/TOPATJ130905003.pdfTest

المؤلفون: Griffiths, William J, Yutuc, Eylan, Abdel-Khalik, Jonas, Crick, Peter J, Hearn, Thomas, Dickson, Alison, Bigger, Brian W, Hoi-Yee Wu, Teresa, Goenka, Anu, Ghosh, Arunabha, Jones, Simon A, Covey, Douglas F, Ory, Daniel S, Wang, Yuqin

المصدر: Griffiths , W J , Yutuc , E , Abdel-Khalik , J , Crick , P J , Hearn , T , Dickson , A , Bigger , B W , Hoi-Yee Wu , T , Goenka , A , Ghosh , A , Jones , S A , Covey , D F , Ory , D S & Wang , Y 2019 , ' Metabolism of Non-Enzymatically Derived Oxysterols : Clues from sterol metabolic disorders ' , Free Radical Biology and Medicine , vol. 144 , pp. 124-133 . https://doi.org/10.1016/j.freeradbiomed.2019.04.020Test

مصطلحات موضوعية: Biotransformation, Cholestanols/blood, Cholesterol/blood, Cholic Acids/biosynthesis, Chromatography, Liquid, Epoxide Hydrolases/blood, Free Radicals/blood, Humans, Hydroxycholesterols/blood, Hydroxylation, Ketocholesterols/blood, Lysosomal Storage Diseases/blood, Mass Spectrometry, Niemann-Pick Diseases/blood, Oxidation-Reduction, Wolman Disease/blood

العلاقة: https://research-portal.st-andrews.ac.uk/en/researchoutput/metabolism-of-nonenzymatically-derived-oxysterolsTest(2605f549-03ca-436b-b3f2-53398944f267).html

الإتاحة: https://doi.org/10.1016/j.freeradbiomed.2019.04.020Test
https://research-portal.st-andrews.ac.uk/en/researchoutput/metabolism-of-nonenzymatically-derived-oxysterolsTest(2605f549-03ca-436b-b3f2-53398944f267).html

المؤلفون: Griffiths, William J, Yutuc, Eylan, Abdel-Khalik, Jonas, Crick, Peter J, Hearn, Thomas, Dickson, Alison, Bigger, Brian W, Hoi-Yee Wu, Teresa, Goenka, Anu, Ghosh, Arunabha, Jones, Simon A, Covey, Douglas F, Ory, Daniel S, Wang, Yuqin

المصدر: Griffiths , W J , Yutuc , E , Abdel-Khalik , J , Crick , P J , Hearn , T , Dickson , A , Bigger , B W , Hoi-Yee Wu , T , Goenka , A , Ghosh , A , Jones , S A , Covey , D F , Ory , D S & Wang , Y 2019 , ' Metabolism of Non-Enzymatically Derived Oxysterols : Clues from sterol metabolic disorders ' , Free Radical Biology and Medicine , vol. 144 , pp. 124-133 . https://doi.org/10.1016/j.freeradbiomed.2019.04.020Test

مصطلحات موضوعية: Biotransformation, Cholestanols/blood, Cholesterol/blood, Cholic Acids/biosynthesis, Chromatography, Liquid, Epoxide Hydrolases/blood, Free Radicals/blood, Humans, Hydroxycholesterols/blood, Hydroxylation, Ketocholesterols/blood, Lysosomal Storage Diseases/blood, Mass Spectrometry, Niemann-Pick Diseases/blood, Oxidation-Reduction, Wolman Disease/blood

الإتاحة: https://doi.org/10.1016/j.freeradbiomed.2019.04.020Test
https://risweb.st-andrews.ac.uk/portal/en/researchoutput/metabolism-of-nonenzymatically-derived-oxysterolsTest(2605f549-03ca-436b-b3f2-53398944f267).html

المؤلفون: Griffiths, William J, Yutuc, Eylan, Abdel-Khalik, Jonas, Crick, Peter J, Hearn, Thomas, Dickson, Alison, Bigger, Brian W, Hoi-Yee Wu, Teresa, Goenka, Anu, Ghosh, Arunabha, Jones, Simon A, Covey, Douglas F, Ory, Daniel S, Wang, Yuqin

المصدر: Griffiths , W J , Yutuc , E , Abdel-Khalik , J , Crick , P J , Hearn , T , Dickson , A , Bigger , B W , Hoi-Yee Wu , T , Goenka , A , Ghosh , A , Jones , S A , Covey , D F , Ory , D S & Wang , Y 2019 , ' Metabolism of Non-Enzymatically Derived Oxysterols : Clues from sterol metabolic disorders ' , Free Radical Biology and Medicine , vol. 144 , pp. 124-133 . https://doi.org/10.1016/j.freeradbiomed.2019.04.020Test

مصطلحات موضوعية: Biotransformation, Cholestanols/blood, Cholesterol/blood, Cholic Acids/biosynthesis, Chromatography, Liquid, Epoxide Hydrolases/blood, Free Radicals/blood, Humans, Hydroxycholesterols/blood, Hydroxylation, Ketocholesterols/blood, Lysosomal Storage Diseases/blood, Mass Spectrometry, Niemann-Pick Diseases/blood, Oxidation-Reduction, Wolman Disease/blood

العلاقة: https://research-portal.st-andrews.ac.uk/en/researchoutput/metabolism-of-nonenzymatically-derived-oxysterolsTest(2605f549-03ca-436b-b3f2-53398944f267).html

الإتاحة: https://doi.org/10.1016/j.freeradbiomed.2019.04.020Test
https://research-portal.st-andrews.ac.uk/en/researchoutput/metabolism-of-nonenzymatically-derived-oxysterolsTest(2605f549-03ca-436b-b3f2-53398944f267).html

المؤلفون: Lund, Frederik W., Wüstner, Daniel

المساهمون: Öllinger, Karin, Appelqvist, Hanna

المصدر: Lund , F W & Wüstner , D 2017 , Quantitative co-localization and pattern analysis of endo-lysosomal cargo in subcellular image cytometry and validation on synthetic image sets . in K Öllinger & H Appelqvist (eds) , Lysosomes : Methods and Protocols . Humana Press , Methods in Molecular Biology , vol. 1594 , pp. 93-128 . https://doi.org/10.1007/978-1-4939-6934-0_6Test

مصطلحات موضوعية: Animals, Cholesterol/metabolism, Endocytosis/physiology, Endosomes/metabolism, Humans, Lysosomal Storage Diseases/metabolism, Lysosomes/metabolism, Microscopy, Fluorescence, Monte Carlo Method, Niemann-Pick Diseases/metabolism, Protein Transport/physiology

العلاقة: https://portal.findresearcher.sdu.dk/da/publications/2aee9e82-bcfc-4811-99b5-976744f8b5c0Test; urn:ISBN:978-1-4939-6932-6

الإتاحة: https://doi.org/10.1007/978-1-4939-6934-0_6Test
https://portal.findresearcher.sdu.dk/da/publications/2aee9e82-bcfc-4811-99b5-976744f8b5c0Test

المؤلفون: Bigger, Brian W, Saif, Muhammad, Linthorst, Gabor E

المصدر: Bigger , B W , Saif , M & Linthorst , G E 2015 , ' The role of antibodies in enzyme treatments and therapeutic strategies. ' , Best practice & research. Clinical endocrinology & metabolism , vol. 29 , no. 2 . https://doi.org/10.1016/j.beem.2015.01.006Test

مصطلحات موضوعية: antibodies, biomarker, lysosomal storage diseases enzyme replacement therapy, response

الإتاحة: https://doi.org/10.1016/j.beem.2015.01.006Test
https://research.manchester.ac.uk/en/publications/176d75cc-86c4-4f85-b218-f4e4ce5da7d4Test

المؤلفون: Detlefsen, Sönke, Fagerberg, Christina R, Ousager, Lilian Bomme, Lindebjerg, Jan, Marcussen, Niels, Nathan, Torben, Sørensen, Flemming B

المصدر: Detlefsen , S , Fagerberg , C R , Ousager , L B , Lindebjerg , J , Marcussen , N , Nathan , T & Sørensen , F B 2013 , ' Histiocytic disorders of the gastrointestinal tract ' , Human Pathology , vol. 44 , no. 5 , pp. 683-696 . https://doi.org/10.1016/j.humpath.2012.05.010Test

مصطلحات موضوعية: Erdheim-Chester disease, Langerhans cell histiocytosis, Malacoplakia, Melanosis coli, Rosai-Dorfman disease, Whipple disease, Xanthogranulomatous inflammation, Xanthomatosis, Immunohistochemistry, Erdheim-Chester Disease/pathology, Malacoplakia/pathology, Humans, Child, Preschool, Male, Whipple Disease/pathology, Xanthomatosis/complications, Young Adult, Tangier Disease/pathology, Adult, Gastrointestinal Diseases/pathology, Histiocytosis, Langerhans-Cell/pathology, Diagnosis, Differential, Lysosomal Storage Diseases/pathology, Sinus/pathology, Histiocytosis/pathology, Xanthogranuloma, Juvenile/pathology

العلاقة: https://portal.findresearcher.sdu.dk/da/publications/469387f3-0cd0-4e78-b285-98a813d05068Test

الإتاحة: https://doi.org/10.1016/j.humpath.2012.05.010Test
https://portal.findresearcher.sdu.dk/da/publications/469387f3-0cd0-4e78-b285-98a813d05068Test

المؤلفون: Abbott, N. Joan

المصدر: Abbott , N J 2013 , ' Blood-brain barrier structure and function and the challenges for CNS drug delivery ' , Journal of Inherited Metabolic Disease , vol. 36 , no. 3 , pp. 437-449 . https://doi.org/10.1007/s10545-013-9608-0Test

مصطلحات موضوعية: CENTRAL-NERVOUS-SYSTEM, LYSOSOMAL STORAGE DISEASES, ENZYME-REPLACEMENT-THERAPY, RECEPTOR-MEDIATED TRANSCYTOSIS, IN-VITRO MODELS, MOUSE MODEL, ENDOTHELIAL-CELLS, METACHROMATIC LEUKODYSTROPHY, NEUROVASCULAR UNIT, ABC TRANSPORTERS

الإتاحة: https://doi.org/10.1007/s10545-013-9608-0Test
https://kclpure.kcl.ac.uk/portal/en/publications/32893c94-a1ff-4733-ab1b-a37cffb912a7Test

المؤلفون: Abbott, N. Joan

المصدر: Abbott , N J 2013 , ' Blood-brain barrier structure and function and the challenges for CNS drug delivery ' , Journal of Inherited Metabolic Disease , vol. 36 , no. 3 , pp. 437-449 . https://doi.org/10.1007/s10545-013-9608-0Test

مصطلحات موضوعية: CENTRAL-NERVOUS-SYSTEM, LYSOSOMAL STORAGE DISEASES, ENZYME-REPLACEMENT-THERAPY, RECEPTOR-MEDIATED TRANSCYTOSIS, IN-VITRO MODELS, MOUSE MODEL, ENDOTHELIAL-CELLS, METACHROMATIC LEUKODYSTROPHY, NEUROVASCULAR UNIT, ABC TRANSPORTERS

الإتاحة: https://doi.org/10.1007/s10545-013-9608-0Test
https://kclpure.kcl.ac.uk/portal/en/publications/bloodbrain-barrier-structure-and-function-and-the-challenges-for-cns-drug-deliveryTest(32893c94-a1ff-4733-ab1b-a37cffb912a7).html

المؤلفون: Wraith, James, Wraith, J. Edmond

المصدر: Wraith , J & Wraith , J E 2008 , ' Enzyme replacement therapy with idursulfase in patients with mucopolysaccharidosis type II ' , Acta Paediatrica, International Journal of Paediatrics , vol. 97 , no. 457 , pp. 76-78 . https://doi.org/10.1111/j.1651-2227.2008.00661.xTest

مصطلحات موضوعية: Enzyme replacement therapy, Hunter syndrome, Idursulfase, Lysosomal storage diseases, Mucopolysaccharidosis type II

الإتاحة: https://doi.org/10.1111/j.1651-2227.2008.00661.xTest
https://research.manchester.ac.uk/en/publications/bca9a647-44d6-4157-8f32-8e2a3b7648fcTest