Results 51 to 60 of about 31,866 (306)

Alterations in hepatic miRNA expression during negative energy balance in postpartum dairy cattle [PDF]

, 2014
peer-reviewedBackground Negative energy balance (NEB), an altered metabolic state, occurs in early postpartum dairy cattle when energy demands to support lactation exceed energy intake.
Fatima, Attia, Morris, Dermot G., O'Boyle, Padraig, Seoighe, Cathal, Waters, Sinead M.   +4 more
core   +1 more source

Iduronate-2-Sulfatase with Anti-human Transferrin Receptor Antibody for Neuropathic Mucopolysaccharidosis II: A Phase 1/2 Trial

Molecular Therapy, 2018
Hunter syndrome (mucopolysaccharidosis II [MPS II]), a deficiency of iduronate-2-sulfatase (IDS), causes an accumulation of glycosaminoglycans, giving rise to multiple systemic and CNS symptoms.
T. Okuyama, Y. Eto, N. Sakai, K. Minami, Tatsuyoshi Yamamoto, Hiroyuki Sonoda, Mariko Yamaoka, K. Tachibana, T. Hirato, Yuji Sato   +9 more
semanticscholar   +1 more source

Distribution of chromaffin secretory vesicles, acetylcholinesterase, and lysosomal enzymes in sucrose and percoll gradients [PDF]

, 1984
Crude chromaffin secretory vesicles, obtained by differential centrifugation, were further purified on isotonic (Percoll) gradients. The chromaffin vesicle fractions recovered from the gradients contain acetylcholinesterase as well as lysosomal enzymes ...
Bowers, Carty, Cheng, Cheng, Chubb, Farquhar, Gratzi, Gratzl, Gratzl, Hall, Hasilik, Jørgensen, Krieger-Brauer, Krieger-Brauer, Krieger-Brauer, Little, Manfred Gratzl, Meisler, Mizobe, Mizobe, Saermark, Saermark, Saermark, Saermark, Schneider, Schneider, Schneider, Silsand, Smith, Terland, Wilson, Zinder   +31 more
core   +1 more source

The Chihuahua dog: A new animal model for neuronal ceroid lipofuscinosis CLN7 disease? [PDF]

, 2016
Neuronal ceroid lipofuscinoses (NCLs) are a group of incurable lysosomal storage disorders characterized by neurodegeneration and accumulation of lipopigments mainly within the neurons. We studied two littermate Chihuahua dogs presenting with progressive
Alroy, Joseph, Anderson, Glenn W., Bras, Jose, Darwent, Lee, Faller, Kiterie M.E., Guerreiro, Rita J., Gutierrez Quintana, Rodrigo, Kun-Rodrigues, Celia, Mole, Sara E., Penderis, Jacques, Sharpe, Samuel J.   +10 more
core   +2 more sources

Molecular analysis of iduronate -2- sulfatase gene in Tunisian patients with mucopolysaccharidosis type II [PDF]

, 2011
Mucopolysaccharidosis type II (MPS II, Hunter syndrome) is X-linked recessive lysosomal storage disorder resulting from the defective activity of the enzyme iduronate-2-sulfatase (IDS).
Latifa Chkioua, Souhir Khedhiri, Salima Ferchichi, Rémy Tcheng, Henda Chahed, Roseline Froissart, Christine Vianey-Saban, Sandrine Laradi, Abdelhedi Miled   +8 more
core   +2 more sources

Sulfatases and sulfatase modifying factors: an exclusive and promiscuous relationship [PDF]

Human Molecular Genetics, 2005
Sulfatases catalyze the hydrolysis of sulfate ester bonds from a wide variety of substrates. Several human inherited diseases are caused by the deficiency of individual sulfatases, while in patients with multiple sulfatase deficiency mutations in the Sulfatase Modifying Factor 1 (SUMF1) gene cause a defect in the post-translational modification of a ...
SARDIELLO M., ANNUNZIATA I., ROMA G., BALLABIO, ANDREA   +3 more
openaire   +3 more sources

Multiple sulfatase deficiency with neonatal manifestation. [PDF]

, 2014
Multiple Sulfatase Deficiency (MSD; OMIM 272200) is a rare autosomal recessive inborn error of metabolism caused by mutations in the sulfatase modifying factor 1 gene, encoding the formylglycine-generating enzyme (FGE), and resulting in tissue ...
Ballabio, A., Bernasconi, S., Braibanti, S., Frattini, D., Gabrielli, O., Galeazzi, T., Garavelli, L., Gargano, G., Gelmini, C., Iori, A., Iughetti, L., Ivanovski, I., Melli, N., Padella, L., Pedori, S., Pepe, S., Rosato, S., Santoro, L., Superti-Furga, A., Wischmeijer, A., Zampini, L.   +20 more
core   +1 more source

SUMF1 enhances sulfatase activitiesin vivoin five sulfatase deficiencies [PDF]

Biochemical Journal, 2007
Sulfatases are enzymes that hydrolyse a diverse range of sulfate esters. Deficiency of lysosomal sulfatases leads to human diseases characterized by the accumulation of either GAGs (glycosaminoglycans) or sulfolipids. The catalytic activity of sulfatases resides in a unique formylglycine residue in their active site generated by the post-translational ...
FRALDI, ALESSANDRO, A. Biffi, A. Lombardi, I. Visigalli, S. Pepe, SETTEMBRE, CARMINE, E. Nusco, AURICCHIO, ALBERTO, L. Naldini, BALLABIO, ANDREA, M. P. C.o.s.m.a.   +10 more
openaire   +4 more sources

Discovery of a novel iota carrageenan sulfatase isolated from the marine bacterium Pseudoalteromonas carrageenovora

Frontiers in Chemistry, 2014
Carrageenans are sulfated polysaccharides extracted from the cell wall of some marine red algae. These polysaccharides are widely used as gelling, stabilizing, and viscosifying agents in the food and pharmaceutical industries.
Sabine Marie Genicot, Sabine Marie Genicot, Agnès eGroisillier, Agnès eGroisillier, Hélène eRogniaux, Laurence eMeslet-Cladière, Laurence eMeslet-Cladière, Laurence eMeslet-Cladière, Tristan eBarbeyron, Tristan eBarbeyron, William eHelbert, William eHelbert, William eHelbert   +12 more
doaj   +1 more source

Proteolytic cleavage of the rat heparan sulfate 6-O-endosulfatase SulfFP2 by furintype proprotein convertases [PDF]

, 2010
journal ...
KEINO-MASU Kazuko, MASU Masayuki, Nagamine Satoshi, Shiomi Kensuke   +3 more
core   +1 more source