Results 61 to 70 of about 60,786 (163)

Extracellularly Activatable Conjugates of RGD Peptidomimetics and Cryptophycin for αVβ3‐Targeted Cancer Therapy

Chemistry – A European Journal, EarlyView.
Integrin αVβ3‐targeting small‐molecule drug conjugates (SMDCs) were synthesized by conjugating a cryptophycin payload through a neutrophil elastase‐cleavable NPV‐PABC linker. RGD peptidomimetic and linker epimers served as controls for targeting and enzymatic cleavage, and the resulting conjugates displayed subnanomolar cytotoxicity in vitro.
Dominic Seißenschmidt, Henriette Hartung, Jonah Kammer, Norbert Sewald   +3 more
wiley   +1 more source

Characterization of Lysosomal Hydrolases and Transporters and Their Age‐Dependent Variability: Relevance to Drug Metabolism and Transport of Small Molecule and Biologic Drugs

Clinical Pharmacology &Therapeutics, EarlyView.
Lysosomes play a key role in the accumulation, catabolism, and transport of endogenous and exogenous metabolites and proteins and are involved in drug metabolism and prodrug activation. However, the protein abundance and interindividual variability of lysosomal drug‐metabolizing enzymes and transporters (DMETs) remain underexplored.
Darshak Gadara, Dilip Kumar Singh, Sandhya Subash, Guihua Yue, Deepak Ahire, Chris Bohl, Maciej Czerwinski, Stephanie Helmstetter, Scott Heyward, Robert S. Jones, Cyrus Khojasteh, Ryota Kikuchi, Priyanka Kulkarni, Bin Ma, Bernard Murray, Chris Seib, Bill Smith, David M. Stresser, Mitchell E. Taub, Ting Wang, Bhagwat Prasad   +20 more
wiley   +1 more source

Heparan sulfate and glycomimetics: Advances in synthesis and biological applications for post‐stroke neurorepair

Neuroprotection, EarlyView.
ECM, extracellular matrix; BDNF, brain‐derived neuroptrophic factor; FGF, fibroblast growth factor; VEGF, vascular endothelial growth factor. Abstract Heparan sulfate, a structurally diverse glycosaminoglycan that is abundant in the central nervous system (CNS), orchestrates essential processes fundamental to neural plasticity, neurorepair, and ...
Mozammel H. Bhuiyan, Madeleine J. Smith, Simon F. R. Hinkley, Andrew N. Clarkson   +3 more
wiley   +1 more source

Multiple sulfatase deficiency in a Turkish family resulting from a novel mutation.

, 2008
Multiple sulfatase deficiency (MSD) is an inherited lysosomal storage disease that affects post-translational activation of all of the sulfatases. Since biochemical and clinical findings are variable, the diagnosis is difficult in most of the cases ...
YİŞ, ULUÇ, BALLABIO, ANDREA, Cosma M. P., Kurul, Semra Hiz, Pepe S., Dirik E., Cosma, Maria Pia, Kurul S. H., Pepe, Stefano, Yiş U., Dirik, Eray   +10 more
core   +1 more source

Structure and functional genes associated with carbon, nitrogen, phosphorus, and sulfur cycling processes in periphyton: A comparison between natural and artificial rivers

River, EarlyView.
Network analysis of functional genes among prokaryotes, and eukaryotes in natural and artificial rivers. Abstract Periphyton in aquatic ecosystems plays vital roles in the elemental cycle process and is vulnerable to anthropogenic interference. However, few studies have explored the elemental cycles of carbon (C), nitrogen (N), phosphorus (P), and ...
Yulu Tian, Jiezhang Mo, Jiaxin Liu, Chenghao Li, Jiahua Guo   +4 more
wiley   +1 more source

A Description of Skeletal Manifestation in Adult Case of Morquio Syndrome: Radiographic and MRI Appearance

Case Reports in Medicine, 2012
We report on a rare case of Morquio syndrome, an autosomal recessive mucopolysaccharidosis including type IVA, a deficiency of N-acetylgalctosamine-6-sulfatase and type IVB a deficiency of β-galactosidase.
Annalisa Di Cesare, Alessandra Di Cagno, Stefano Moffa, Paolucci Teresa, Innocenzi Luca, Arrigo Giombini   +5 more
doaj   +1 more source

Mucopolysaccharidosis type II: European recommendations for the diagnosis and multidisciplinary management of a rare disease

Orphanet Journal of Rare Diseases, 2011
Mucopolysaccharidosis type II (MPS II) is a rare, life-limiting, X-linked recessive disease characterised by deficiency of the lysosomal enzyme iduronate-2-sulfatase. Consequent accumulation of glycosaminoglycans leads to pathological changes in multiple
Scarpa Maurizio, Almássy Zsuzsanna, Beck Michael, Bodamer Olaf, Bruce Iain A, De Meirleir Linda, Guffon Nathalie, Guillén-Navarro Encarna, Hensman Pauline, Jones Simon, Kamin Wolfgang, Kampmann Christoph, Lampe Christina, Lavery Christine A, Leão Teles Elisa, Link Bianca, Lund Allan M, Malm Gunilla, Pitz Susanne, Rothera Michael, Stewart Catherine, Tylki-Szymańska Anna, van der Ploeg Ans, Walker Robert, Zeman Jiri, Wraith James E   +25 more
doaj   +1 more source

RNF13 is a previously undescribed interactor of iduronate 2‐sulfatase that modifies its glycosylation and maturation

The FEBS Journal, EarlyView.
Iduronate 2‐sulfatase (IDS; purple) is expressed as a precursor protein that goes through multiple steps of maturation, modification, and trafficking to become an active lysosomal enzyme that degrades glycosaminoglycans. Our study shows that the transmembrane ubiquitin ligases RNF13 (orange) and RNF167 (pink) heterodimerize, affecting IDS intracellular
Valérie C. Cabana, Antoine Y. Bouchard, Audrey M. Sénécal, Laurent Cappadocia, Marc P. Lussier   +4 more
wiley   +1 more source

Natural history of Morquio A patient with tracheal obstruction from birth to death

Molecular Genetics and Metabolism Reports, 2018
Morquio A syndrome (mucopolysaccharidosis IVA, MPS IVA) is a lysosomal storage disease caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase, resulting in systemic accumulation of the partially degraded glycosaminoglycans (GAGs), keratan ...
Caitlin Doherty, Lauren W. Averill, Mary Theroux, William G. Mackenzie, Christian Pizarro, Robert W. Mason, Shunji Tomatsu   +6 more
doaj   +1 more source

Steroidogenic compensation and lipid deficiency with enhanced NAD+ salvage in small‐for‐gestational‐age placenta

The FEBS Journal, EarlyView.
Fetal growth restriction is associated with placental metabolic adaptations. In small‐for‐gestational‐age placenta (SGA), cholesterol receptors and steroidogenic enzymes are upregulated, enhancing steroidogenesis. NAD salvage pathway is also increased to support NADP+/NADPH requirements.
Serena Xodo, Lorenza Driul, Vanessa Tolotto, Eros Di Giorgio, Luigi E. Xodo   +4 more
wiley   +1 more source