Results 101 to 110 of about 2,680,532 (210)

Human acid sphingomyelinase structures provide insight to molecular basis of Niemann–Pick disease

Nature Communications, 2016
Genetic alterations in the protein acid sphingomyelinase (ASM) lead to ASM deficiency and have been associated with Niemann–Pick disease. Here, the authors report the crystal structures of ASM alone and bound to its product, and discuss the catalytic ...
Yan-Feng Zhou, Matthew C. Metcalf, Scott C. Garman, Tim Edmunds, Huawei Qiu, Ronnie R. Wei   +5 more
doaj   +1 more source

Kinetics of acid sphingomyelinase serum activity in the control group.

, 2014
The box plots display the minima/maxima (whiskers), 25%/75% percentile (box), median (−) and mean (+) values.
Supandi Winoto-Morbach (501243), Matthias Kott (657467), Gunnar Elke (449417), Inéz Frerichs (20627), Norbert Weiler (20632), Stefan Schütze (320441), Günther Zick (20626), Maike Reinicke (657468), Dirk Schädler (20628)   +8 more
core   +1 more source

Acid sphingomyelinase deals the seal

Journal of Cell Biology, 2010
Cells repair holes in their plasma membrane by secreting a lysosomal hydrolase to induce endocytosis, [Tam et al.][1] reveal. ![Figure][2] When ASM is inhibited (right), the holes produced by a bacterial toxin aren't resealed, allowing a lipophilic dye (green) to enter the cells.
openaire   +2 more sources

Fluoxetine and Sertraline Potently Neutralize the Replication of Distinct SARS-CoV-2 Variants

Viruses
The pandemic caused by SARS-CoV-2 is still a major health problem. Newly emerging variants and long-COVID-19 represent a challenge for the global health system.
Laura Thümmler, Nadine Beckmann, Carolin Sehl, Matthias Soddemann, Peer Braß, Maren Bormann, Leonie Brochhagen, Carina Elsner, Nicolas Hoertel, Céline Cougoule, Sandra Ciesek, Marek Widera, Ulf Dittmer, Monika Lindemann, Peter A. Horn, Oliver Witzke, Stephanie Kadow, Markus Kamler, Erich Gulbins, Katrin Anne Becker, Adalbert Krawczyk   +20 more
doaj   +1 more source

The impact of sphingomyelin on the pathophysiology and treatment response to olipudase alfa in acid sphingomyelinase deficiency

Genetics in Medicine Open
Acid sphingomyelinase deficiency (ASMD) is a rare progressive genetic disorder caused by pathogenic variants in the SMPD1 gene causing low or absent activity of the enzyme acid sphingomyelinase, resulting in subsequent accumulation of its substrate ...
Monica Kumar, Mario Aguiar, Andreas Jessel, Beth L. Thurberg, Lisa Underhill, Holly Wong, Kelly George, Vanessa Davidson, Edward H. Schuchman   +8 more
doaj   +1 more source

Favorable clinical response to olipudase alfa in a patient with two variants of uncertain significance in SMPD1: a case report

Egyptian Journal of Medical Human Genetics
Background Acid sphingomyelinase deficiency (ASMD), also known as Niemann–Pick disease, is a rare lysosomal storage disorder caused by biallelic pathogenic variants in the Sphingomyelin phosphodiesterase 1 (SMPD1) gene, leading to the deficient activity ...
Luz María Sánchez-Sánchez, Jorge Álvarez-Longoria, Beatriz De la Fuente-Cortez, Valentina Martínez-Montoya, Ekaterina Sánchez-Romero   +4 more
doaj   +1 more source

The relationship of sphingolipid mechanisms with oxidative stress and changes in mitochondria during functional unloading of postural muscles

Acta Biomedica Scientifica
Background. Prolonged inactivity of skeletal muscles is accompanied by the development of oxidative stress and changes in sphingolipid metabolism. The relationship of sphingolipid mechanisms with generation of reactive oxygen species (ROS) in muscles ...
V. A. Protopopov, A. V. Sekunov, A. V. Panov, I. G. Bryndina   +3 more
doaj   +1 more source

Targeting acid sphingomyelinase reduces cardiac ceramide accumulation in the post-ischemic heart

, 2016
Ceramide accumulation is known to accompany acute myocardial ischemia, but its role in the pathogenesis of ischemic heart disease is unclear. In this study, we aimed to determine how ceramides accumulate in the ischemic heart and to determine if cardiac ...
Andersson, Linda, Scharin T\ue4ng, Margareta, Lundqvist, Annika,, Adiels, Martin,, Adiels, Martin, Ståhlman, Marcus,, Fogelstrand, Per,, Levin, Malin,, Klevstig, Martina,, Klevstig, M., Andersson, Linda,, Bor\ue9n, Jan, Fogelstrand, Per, Jeppsson, Anders, Lundqvist, Annika, Scharin Täng, Margareta,, Borén, Jan,, Levin, Malin, St\ue5hlman, Marcus, Kolesnick, R., Jeppsson, Anders,, Klevstig, M.,   +21 more
core   +1 more source

The steady-state level of plasma membrane ceramide is regulated by neutral sphingomyelinase 2

Journal of Lipid Research
During the last 30 years, an increasing number of cellular functions have been reported to be regulated by the lipid ceramide. The diversity in the ceramide structure, leading to tens of ceramide species and the discrete distribution based on subcellular
Anne G. Ostermeyer-Fay, Abhay Kanodia, Ranjana Pathak, Maria Jose Hernandez-Corbacho, Aarnoud C. van der Spoel, Yusuf A. Hannun, Daniel Canals   +6 more
doaj   +1 more source

Acid Sphingomyelinase Inhibition Prevents Development of Sepsis Sequelae in the Murine Liver

, 2017
The molecular mechanisms of maladaptive response in liver tissue with respect to the acute and post-acute phase of sepsis are not yet fully understood. Long-term sepsis survivors might develop hepatocellular/hepatobiliary injury and fibrosis.
Falk A. Gonnert, Benjamin Giszas, Jorge Hurtado-Oliveros, C. Julius Witt, Ralf A. Claus, Ha-Yeun Chung, Tony Bruns, Andreas Stallmach, Amelie Lupp, Nayla Jbeily, Markus H. Gräler   +10 more
core   +1 more source