Results 101 to 110 of about 5,986,972 (170)

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

FARBER DISEASE — DISEASE DESCRIPTION WITH CASE REPORTS

Вопросы современной педиатрии, 2014
Farber disease (lipogranulomatosis, OMIM 228000) — is extremely rare autosomal-recessive disorder from group of lysosomal storage disorders, due to deficiency of acid ceramidase activity enzyme.
I. A. Chikova, N. V. Buchinskaya, М. М. Kostik, V. V. Avramenko, O. L. Krasnogorskaya, R. A. Nasirov, T. Levade, V. G. Chasnyk   +7 more
doaj   +1 more source

C26-Ceramide as highly sensitive biomarker for the diagnosis of Farber Disease

Scientific Reports, 2017
Farber disease (FD) is a rare autosomal recessive disease caused by mutations in the acid ceramidase gene (ASAH1). Low ceramidase activity results in the accumulation of fatty substances, mainly ceramides.
Claudia Cozma, Marius-Ionuț Iurașcu, Sabrina Eichler, Marina Hovakimyan, Oliver Brandau, Susanne Zielke, Tobias Böttcher, Anne-Katrin Giese, Jan Lukas, Arndt Rolfs   +9 more
doaj   +1 more source

A simple fluorogenic method for determination of acid ceramidase activity and diagnosis of Farber disease

Journal of Lipid Research, 2010
Acid ceramidase (aCDase) is one of several enzymes responsible for ceramide degradation within mammalian cells. As such, aCDase regulates the intracellular levels of the bioactive lipid ceramide.
Carmen Bedia, Luz Camacho, José Luís Abad, Gemma Fabriàs, Thierry Levade   +4 more
doaj   +1 more source

Mitochondrial Cholesterol in Alzheimer's Disease and Niemann–Pick Type C Disease

Frontiers in Neurology, 2019
Mitochondrial dysfunction has been recognized as a key player in neurodegenerative diseases, including Alzheimer's disease (AD) and Niemann–Pick type C (NPC) disease.
Sandra Torres, Sandra Torres, Carmen M. García-Ruiz, Carmen M. García-Ruiz, Carmen M. García-Ruiz, Jose C. Fernandez-Checa, Jose C. Fernandez-Checa, Jose C. Fernandez-Checa   +7 more
doaj   +1 more source

Human Acid Ceramidase [PDF]

Journal of Biological Chemistry, 2001
Klaus Ferlinz, Guido Kopal, Katussevani Bernardo, Thomas Linke, Julia Bär, Bernadette Breiden, Ulrich Neumann, Florian Lang, Edward H. Schuchman, Konrad Sandhoff   +9 more
openaire   +1 more source

Use of Acid Ceramidase and Sphingosine Kinase Inhibitors as Antiviral Compounds Against Measles Virus Infection of Lymphocytes in vitro

Frontiers in Cell and Developmental Biology, 2019
As structural membrane components and signaling effector molecules sphingolipids influence a plethora of host cell functions, and by doing so also the replication of viruses.
Anika Grafen, Fabian Schumacher, Fabian Schumacher, Janice Chithelen, Burkhard Kleuser, Niklas Beyersdorf, Jürgen Schneider-Schaulies   +6 more
doaj   +1 more source

Ceramide generation as a novel biological mechanism for chemo-preventive and cytotoxic effects of hesperidin on HT-144 melanoma cells

Beni-Suef University Journal of Basic and Applied Sciences, 2018
Introduction: Hesperidin and its aglycone, hesperetin are abundant natural flavonoid in Citrus and known for their anti-neoplastic and pro-apoptotic properties.
Mahdi Mashhadi Akbar Boojar, Masoud Mashhadi Akbar Boojar, Sepide Golmohammad, Mojtaba Nikkhah Yazdi   +3 more
doaj   +1 more source

Genetic Variation in Acid Ceramidase Predicts Non-completion of an Exercise Intervention

Frontiers in Physiology, 2018
Genetic variation is associated with a number of lifestyle behaviours; it may be associated with adherence and individual responses to exercise training. We tested single nucleotide polymorphisms (SNPs) in the acid ceramidase gene (ASAH1) for association
Lauren S. Lewis, Kim M. Huffman, Kim M. Huffman, Ira J. Smith, Mark P. Donahue, Cris A. Slentz, Joseph A. Houmard, Monica J. Hubal, Eric P. Hoffman, Elizabeth R. Hauser, Elizabeth R. Hauser, Ilene C. Siegler, William E. Kraus, William E. Kraus   +13 more
doaj   +1 more source

Activity-Based Imaging of Acid Ceramidase in Living Cells

Journal of the American Chemical Society, 2019
Acid ceramidase (AC) hydrolyzes ceramides into sphingoid bases and fatty acids. The enzyme is overexpressed in several types of cancer and Alzheimer's disease, and its genetic defect causes different incurable disorders. The availability of a method for the specific visualization of catalytically active AC in intracellular compartments is crucial for ...
Ordóñez, Yadira, Abad, José, Aseeri, Mazen, Casas, Josefina, Garcia, Virginie, Casasampere, Mireia, Schuchman, Edward, Levade, Thierry, Delgado, Antonio, Triola, Gemma, Fabrias, Gemma   +10 more
openaire   +3 more sources