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Activation of Nrf2 in keratinocytes causes chloracne (MADISH)‐like skin disease in mice

Schäfer, Matthias; Willrodt, Ann‐helen; Kurinna, Svitlana; Link, Andrea S; Farwanah, Hany; Geusau, Alexandra; Gruber, Florian; Sorg, Olivier; Huebner, Aaron J; Roop, Dennis; Sandhoff, Konrad; Saurat, Jean‐hilaire; Tschachler, Erwin; Schneider, Marlon R; Langbein, Lutz; Bloch, Wilhelm; Beer, Hans‐dietmar; Werner, Sabine (EMBO Press, 2014)

The transcription factor Nrf2 is a key regulator of the cellular stress response, and pharmacological Nrf2 activation is a promising strategy for skin protection and cancer prevention. We show here that prolonged Nrf2 ...

CLN3 loss disturbs membrane microdomain properties and protein transport in brain endothelial cells

Tecedor, Luis; Stein, Colleen S; Schultz, Mark L; Farwanah, Hany; Sandhoff, Konrad; Davidson, Beverly L (Society for Neuroscience, 2013)

Juvenile neuronal ceroid lipofuscinosis (JNCL) is a fatal childhood-onset neurodegenerative disorder caused by mutations in ceroid lipofuscinosis neuronal-3 (CLN3), a hydrophobic transmembrane protein of unresolved function. ...

Loss of CB1 receptors leads to decreased cathepsin D levels and accelerated lipofuscin accumulation in the hippocampus

Piyanova, Anastasia; Albayram, Onder; Rossi, Carlo Alberto; Farwanah, Hany; Michel, Kerstin; Nicotera, Pierluigi; Sandhoff, Konrad; Bilkei-Gorzo, Andras (Elsevier, 2013)

Early onset of age-related changes in the brain of cannabinoid 1 receptor knockout (Cnr1−/−) mice suggests that cannabinoid 1 (CB1) receptor activity significantly influences the progression of brain aging. In the present ...

Nrf2 links epidermal barrier function with antioxidant defense

Schäfer, Matthias; Farwanah, Hany; Willrodt, Ann‐helen; Huebner, Aaron J; Sandhoff, Konrad; Roop, Dennis; Hohl, Daniel; Bloch, Wilhelm; Werner, Sabine (EMBO Press, 2012)

The skin provides an efficient permeability barrier and protects from microbial invasion and oxidative stress. Here, we show that these essential functions are linked through the Nrf2 transcription factor. To test the ...

Ablation of neuronal ceramide synthase 1 in mice decreases ganglioside levels and expression of myelin-associated glycoprotein in oligodendrocytes

Ginkel, Christina; Hartmann, Dieter; Dorp, Katharina Vom; Zlomuzica, Armin; Farwanah, Hany; Eckhardt, Matthias; Sandhoff, Roger; Degen, Joachim; Rabionet, Mariona; Dere, Ekrem; Dörmann, Peter; Sandhoff, Konrad; Willecke, Klaus (American Society for Biochemistry and Molecular Biology, 2012)

Background: Ceramide synthase 1 catalyzes the synthesis of C18 ceramide and is mainly expressed in neurons of the brain. Results: Ablation of Ceramide Synthase 1 decreases ganglioside levels and expression of oligodendrocytic ...

Mass spectrometric analysis of neutral sphingolipids: methods, applications, and limitations

Farwanah, Hany; Kolter, Thomas; Sandhoff, Konrad (Elsevier, 2011)

Sphingolipids represent an important class among lipids, especially when considering their vital roles in lipid metabolism. Thus, a variety of methods have been created to accomplish their analysis and the term “sphingolipidomics” ...

Schlank, a member of the ceramide synthase family controls growth and body fat in Drosophila

Bauer, Reinhard; Voelzmann, André; Breiden, Bernadette; Schepers, Ute; Farwanah, Hany; Hahn, Ines; Eckardt, Franka; Sandhoff, Konrad; Hoch, Michael (EMBO Press, 2009)

Ceramide synthases are highly conserved transmembrane proteins involved in the biosynthesis of sphingolipids, which are essential structural components of eukaryotic membranes and can act as second messengers regulating ...

Adult ceramide synthase 2 (CERS2)-deficient mice exhibit myelin sheath defects, cerebellar degeneration, and hepatocarcinomas

Imgrund, Silke; Hartmann, Dieter; Farwanah, Hany; Eckhardt, Matthias; Sandhoff, Roger; Degen, Joachim; Gieselmann, Volkmar; Sandhoff, Konrad; Willecke, Klaus (American Society for Biochemistry and Molecular Biology, 2009)

(Dihydro) ceramide synthase 2 (cers2, formerly called lass2) is the most abundantly expressed member of the ceramide synthase gene family, which includes six isoforms in mice. CERS2 activity has been reported to be specific ...

Normal phase liquid chromatography coupled to quadrupole time of flight atmospheric pressure chemical ionization mass spectrometry for separation, detection and mass …

Farwanah, Hany; Wirtz, Jennifer; Kolter, Thomas; Raith, Klaus; Neubert, Reinhard HH; Sandhoff, Konrad (Elsevier, 2009)

Many lipidomic approaches focus on investigating aspects of sphingolipid metabolism. Special emphasis is put on neutral sphingolipids and cholesterol and their interaction. Such an interest is attributed to the fact that ...

Separation and mass spectrometric characterization of covalently bound skin ceramides using LC/APCI-MS and Nano-ESI-MS/MS

Farwanah, Hany; Pierstorff, Barbara; Schmelzer, Christian EH; Raith, Klaus; Neubert, Reinhard HH; Kolter, Thomas; Sandhoff, Konrad (Elsevier, 2007)

Ceramides covalently bound to keratinocytes are essential for the barrier function of the skin, which can be disturbed in diseases, such as psoriasis and atopic dermatitis. These ceramides of the classes ω-hydroxyacyl-sphingosine ...