Results 81 to 90 of about 9,009 (166)

Avaliação de dois anos de tratamento da doença de Gaucher tipo 1 com terapia de reposição enzimática em pacientes do estado de São Paulo, Brasil Evaluation of two years of treatment with enzyme replacement therapy in type 1 Gaucher disease patients of São Paulo State, Brazil

Revista Brasileira de Hematologia e Hemoterapia, 2008
A doença de Gaucher tipo 1 é a doença de depósito lisossômico mais freqüente. De herança autossômica recessiva, é caracterizada pela deficiência da atividade da enzima glicocerebrosidase e o acúmulo patológico de seu substrato, a glicosilceramida, nas ...
Elisa A. P. Sobreira, Paula Bruniera
doaj   +1 more source

Characterization and direct quantitation of cerebroside molecular species from lipid extracts by shotgun lipidomics

Journal of Lipid Research, 2005
By using shotgun lipidomics based on the separation of lipid classes in the electrospray ion source (intrasource separation) and two-dimensional (2D) MS techniques (Han, X., and R. W. Gross. 2004.
Xianlin Han, Hua Cheng
doaj   +1 more source

Epidermal sphingomyelins are precursors for selected stratum corneum ceramides

Journal of Lipid Research, 2000
Epidermal ceramides (Cer) comprise a heterogeneous family of seven species, including two unique ω-hydroxylated Cer, that are key components of the stratum corneum (SC) intercellular lamellar membranes responsible for the epidermal permeability barrier ...
Yoshikazu Uchida, Mariko Hara, Hiroyuki Nishio, Ellen Sidransky, Shintaro Inoue, Fujio Otsuka, Akemi Suzuki, Peter M. Elias, Walter M. Holleran, Sumiko Hamanaka   +9 more
doaj   +1 more source

Membrane Sphingolipids Regulate the Fitness and Antifungal Protein Susceptibility of Neurospora crassa

Frontiers in Microbiology, 2019
The membrane sphingolipid glucosylceramide (GlcCer) plays an important role in fungal fitness and adaptation to most diverse environments. Moreover, reported differences in the structure of GlcCer between fungi, plants and animals render this pathway a ...
Anna Huber, Gregor Oemer, Nermina Malanovic, Karl Lohner, Laura Kovács, Willi Salvenmoser, Johannes Zschocke, Markus A. Keller, Florentine Marx   +8 more
doaj   +1 more source

Glycosphingolipid synthesis requires FAPP2 transfer of glucosylceramide

Nature, 2007
The molecular machinery responsible for the generation of transport carriers moving from the Golgi complex to the plasma membrane relies on a tight interplay between proteins and lipids. Among the lipid-binding proteins of this machinery, we previously identified the four-phosphate adaptor protein FAPP2, the pleckstrin homology domain of which binds ...
D'Angelo, Giovanni, Polishchuk, Elena, Tullio, Giuseppe Di, Santoro, Michele, Campli, Antonella Di, Godi, Anna, West, Gun, Bielawski, Jacek, Chuang, Chia Chen, Van Der Spoel, Aarnoud C., Platt, Frances M., Hannun, Yusuf A., Polishchuk, Roman, Mattjus, Peter, DE MATTEIS, Maria Antonietta   +14 more
openaire   +4 more sources

Diastereomer-specific quantification of bioactive hexosylceramides from bacteria and mammals[S]

Journal of Lipid Research, 2017
Mammals synthesize, cell-type specifically, the diastereomeric hexosylceramides, β-galactosylceramide (GalCer) and β-glucosylceramide (GlcCer), which are involved in several diseases, such as sphingolipidosis, diabetes, chronic kidney diseases, or cancer.
Johanna von Gerichten, Kerstin Schlosser, Dominic Lamprecht, Ivan Morace, Matthias Eckhardt, Dagmar Wachten, Richard Jennemann, Hermann-Josef Gröne, Matthias Mack, Roger Sandhoff   +9 more
doaj   +1 more source

Glucosylceramide synthase inhibitor ameliorates chronic inflammatory pain

Journal of Pharmacological Sciences
Gangliosides play pivotal roles in neuronal tissue processes, such as axonal elongation, synaptic transmission, and neuronal degeneration. Several studies have shown that mice injected with gangliosides synthesized from glucosylceramide exhibit mechanical allodynia.
Shun Watanabe, Misa Oyama, Takashi Iwai, Mitsuo Tanabe   +3 more
openaire   +2 more sources

Drosophila Glucosylceramide Synthase [PDF]

Journal of Biological Chemistry, 2004
Ayako Kohyama-Koganeya, Takeshi Sasamura, Eriko Oshima, Emiko Suzuki, Shoko Nishihara, Ryu Ueda, Yoshio Hirabayashi   +6 more
openaire   +1 more source

Glucosylceramides and kidney disease

, 2017
Glucosylceramides are part of the wider family of glycosylceramides. Glucosylceramide synthase catalyzes the incorporation of a single glucose residue into ceramide to yield glucosylceramide. Glucosylceramide is known as a precursor for globotriaosylceramide (Gb3). In the cell membrane, Gb3 acts a receptor for verotoxin and plays a key role in allowing
Ortiz, Alberto, Sanchez-Nino, Maria Dolores   +1 more
openaire   +1 more source

Sphingolipid profiling reveals differential functions of sphingolipid biosynthesis isozymes of Caenorhabditis elegans

Journal of Lipid Research
Multiple isozymes are encoded in the Caenorhabditis elegans genome for the various sphingolipid biosynthesis reactions, but the contributions of individual isozymes are characterized only in part. We developed a simple but effective reversed-phase liquid
Hui Luo, Xue Zhao, Zi-Dan Wang, Gang Wu, Yu Xia, Meng-Qiu Dong, Yan Ma   +6 more
doaj   +1 more source