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Konrad Sandhoff

Showing results (111-120 of 121) with videos related to

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American Journal of Medical Genetics. Part A|April 27, 2004
Severe subacute GM2 gangliosidosis caused by an apparently silent HEXA mutation (V324V) that results in aberrant splicing and reduced HEXA mRNABrandy A Wicklow, Jennifer L Ivanovich, Margot M Plews, et al.
The Journal of Biological Chemistry|September 15, 2009
Prion-induced activation of cholesterogenic gene expression by Srebp2 in neuronal cellsChristian Bach, Sabine Gilch, Romina Rost, et al.
Nature|January 30, 2010
Hsp70 stabilizes lysosomes and reverts Niemann-Pick disease-associated lysosomal pathologyThomas Kirkegaard, Anke G Roth, Nikolaj H T Petersen, et al.
Genomics|February 7, 2002
Insertional mutagenesis of the mouse acid ceramidase gene leads to early embryonic lethality in homozygotes and progressive lipid storage disease in heterozygotesChi-Ming Li, Jae-Ho Park, Calogera M Simonaro, et al.
Nature Communications|January 20, 2011
PAR2 absence completely rescues inflammation and ichthyosis caused by altered CAP1/Prss8 expression in mouse skinSimona Frateschi, Eric Camerer, Giovanna Crisante, et al.
The Journal of Biological Chemistry|March 5, 2017
Identification of a feedback loop involving β-glucosidase 2 and its product sphingosine sheds light on the molecular mechanisms in Gaucher diseaseSophie Schonauer, Heinz G Körschen, Anke Penno, et al.
Angewandte Chemie (International Ed. in English)|April 6, 2017
Synthetic Glycoforms Reveal Carbohydrate-Dependent Bioactivity of Human Saposin DChristopher G F Graf, Christian Schulz, Marina Schmälzlein, et al.
Orphanet Journal of Rare Diseases|September 28, 2013
Functional and genetic characterization of the non-lysosomal glucosylceramidase 2 as a modifier for Gaucher diseaseYildiz Yildiz, Per Hoffmann, Stefan Vom Dahl, et al.
Journal of Lipid Research|August 3, 2005
A novel mass spectrometric assay for the cerebroside sulfate activator protein (saposin B) and arylsulfatase AAndrew J Norris, Julian P Whitelegge, Arman Yaghoubian, et al.
EMBO Molecular Medicine|February 8, 2014
Activation of Nrf2 in keratinocytes causes chloracne (MADISH)-like skin disease in miceMatthias Schäfer, Ann-Helen Willrodt, Svitlana Kurinna, et al.
Pageof 13

Showing results (111-120 of 121) with videos related to

Sort By:
Pageof 13
American Journal of Medical Genetics. Part A|April 27, 2004
Severe subacute GM2 gangliosidosis caused by an apparently silent HEXA mutation (V324V) that results in aberrant splicing and reduced HEXA mRNABrandy A Wicklow, Jennifer L Ivanovich, Margot M Plews, et al.
The Journal of Biological Chemistry|September 15, 2009
Prion-induced activation of cholesterogenic gene expression by Srebp2 in neuronal cellsChristian Bach, Sabine Gilch, Romina Rost, et al.
Nature|January 30, 2010
Hsp70 stabilizes lysosomes and reverts Niemann-Pick disease-associated lysosomal pathologyThomas Kirkegaard, Anke G Roth, Nikolaj H T Petersen, et al.
Genomics|February 7, 2002
Insertional mutagenesis of the mouse acid ceramidase gene leads to early embryonic lethality in homozygotes and progressive lipid storage disease in heterozygotesChi-Ming Li, Jae-Ho Park, Calogera M Simonaro, et al.
Nature Communications|January 20, 2011
PAR2 absence completely rescues inflammation and ichthyosis caused by altered CAP1/Prss8 expression in mouse skinSimona Frateschi, Eric Camerer, Giovanna Crisante, et al.
The Journal of Biological Chemistry|March 5, 2017
Identification of a feedback loop involving β-glucosidase 2 and its product sphingosine sheds light on the molecular mechanisms in Gaucher diseaseSophie Schonauer, Heinz G Körschen, Anke Penno, et al.
Angewandte Chemie (International Ed. in English)|April 6, 2017
Synthetic Glycoforms Reveal Carbohydrate-Dependent Bioactivity of Human Saposin DChristopher G F Graf, Christian Schulz, Marina Schmälzlein, et al.
Orphanet Journal of Rare Diseases|September 28, 2013
Functional and genetic characterization of the non-lysosomal glucosylceramidase 2 as a modifier for Gaucher diseaseYildiz Yildiz, Per Hoffmann, Stefan Vom Dahl, et al.
Journal of Lipid Research|August 3, 2005
A novel mass spectrometric assay for the cerebroside sulfate activator protein (saposin B) and arylsulfatase AAndrew J Norris, Julian P Whitelegge, Arman Yaghoubian, et al.
EMBO Molecular Medicine|February 8, 2014
Activation of Nrf2 in keratinocytes causes chloracne (MADISH)-like skin disease in miceMatthias Schäfer, Ann-Helen Willrodt, Svitlana Kurinna, et al.
Pageof 13