Konrad Sandhoff

Research interests

Publications

• Gangliosides and Gangliosidoses: Principles of Molecular and Metabolic Pathogenesis

  Journal of Neuroscience · 2013 · 10.1523/jneurosci.0822-13.2013

• Hsp70 stabilizes lysosomes and reverts Niemann–Pick disease-associated lysosomal pathology

  Nature · 2010 · https://doi.org/10.1038/nature08710

• Thalassemia Syndromes

  Journal · 2009 · https://doi.org/10.1007/978-3-540-29676-8_1731

• Adult Ceramide Synthase 2 (CERS2)-deficient Mice Exhibit Myelin Sheath Defects, Cerebellar Degeneration, and Hepatocarcinomas

  Journal of Biological Chemistry · 2009 · 10.1074/jbc.m109.031971

• Direct observation of the nanoscale dynamics of membrane lipids in a living cell

  Nature · 2008 · https://doi.org/10.1038/nature07596

• Apoptotic Vesicles Crossprime CD8 T Cells and Protect against Tuberculosis

  Immunity · 2006 · 10.1016/j.immuni.2005.12.001

• Sphingolipid metabolism diseases

  Biochimica et Biophysica Acta (BBA) - Biomembranes · 2006 · 10.1016/j.bbamem.2006.05.027

• PRINCIPLES OF LYSOSOMAL MEMBRANE DIGESTION: Stimulation of Sphingolipid Degradation by Sphingolipid Activator Proteins and Anionic Lysosomal Lipids

  Annual Review of Cell and Developmental Biology · 2005 · 10.1146/annurev.cellbio.21.122303.120013

• Enhanced insulin sensitivity in mice lacking ganglioside GM3

  Proceedings of the National Academy of Sciences · 2003 · https://doi.org/10.1073/pnas.0635898100

• Combinatorial Ganglioside Biosynthesis

  Journal of Biological Chemistry · 2002 · 10.1074/jbc.r200001200

• CD95 Signaling via Ceramide-rich Membrane Rafts

  Journal of Biological Chemistry · 2001 · https://doi.org/10.1074/jbc.m101207200

• Physiology and pathophysiology of sphingolipid metabolism and signaling

  Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids · 2000 · 10.1016/s1388-1981(00)00042-1

• Sphingolipids—Their Metabolic Pathways and the Pathobiochemistry of Neurodegenerative Diseases

  Angewandte Chemie International Edition · 1999 · 10.1002/(sici)1521-3773(19990601)38:11<1532::aid-anie1532>3.0.co;2-u

• A vital role for glycosphingolipid synthesis during development and differentiation

  Proceedings of the National Academy of Sciences · 1999 · https://doi.org/10.1073/pnas.96.16.9142

• Characterization of Ceramide Synthesis

  Journal of Biological Chemistry · 1997 · 10.1074/jbc.272.36.22432

• Acidic Sphingomyelinase Mediates Entry of N. gonorrhoeae into Nonphagocytic Cells

  Cell · 1997 · 10.1016/s0092-8674(00)80448-1

• Mouse models of Tay–Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism

  Nature Genetics · 1995 · 10.1038/ng1095-170

• Acid sphingomyelinase deficient mice: a model of types A and B Niemann–Pick disease

  Nature Genetics · 1995 · 10.1038/ng0795-288

• Fumonisin B1 inhibits sphingosine (sphinganine) N-acyltransferase and de novo sphingolipid biosynthesis in cultured neurons in situ.

  Journal of Biological Chemistry · 1993 · 10.1016/s0021-9258(19)74249-5

• Ganglioside metabolism. Enzymology, Topology, and regulation.

  Journal of Biological Chemistry · 1993 · 10.1016/s0021-9258(18)53324-x

• Quantitative correlation between the residual activity of β-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease

  Human Genetics · 1992 · 10.1007/bf00219337

• Subcellular localization and membrane topology of serine palmitoyltransferase, 3-dehydrosphinganine reductase, and sphinganine N-acyltransferase in mouse liver.

  Journal of Biological Chemistry · 1992 · 10.1016/s0021-9258(19)49887-6

• AB variant of infantile GM2 gangliosidosis: deficiency of a factor necessary for stimulation of hexosaminidase A-catalyzed degradation of ganglioside GM2 and glycolipid GA2.

  Proceedings of the National Academy of Sciences · 1978 · 10.1073/pnas.75.8.3979

• ENZYME ALTERATIONS AND LIPID STORAGE IN THREE VARIANTS OF TAY‐SACHS DISEASE

  Journal of Neurochemistry · 1971 · 10.1111/j.1471-4159.1971.tb00204.x

• Deficient hexosaminidase activity in an exceptional case of Tay-Sachs disease with additional storage of kidney globoside in visceral organs

  Life Sciences · 1968 · 10.1016/0024-3205(68)90024-6

Current projects

No projects listed.