Results 11 to 20 of about 105,492 (149)

The clinical spectrum of SMA‐PME and in vitro normalization of its cellular ceramide profile

Annals of Clinical and Translational Neurology, Volume 9, Issue 12, Page 1941-1952, December 2022., 2022
Abstract Objective The objectives of this study were to define the clinical and biochemical spectrum of spinal muscular atrophy with progressive myoclonic epilepsy (SMA‐PME) and to determine if aberrant cellular ceramide accumulation could be normalized by enzyme replacement.
Michelle M. Lee, Graeme S. V. McDowell, Darryl C. De Vivo, Daniel Friedman, Samuel F. Berkovic, Maria Spanou, Argirios Dinopoulos, Katheryn Grand, Pedro A. Sanchez‐Lara, Michelle Allen‐Sharpley, Jodi Warman‐Chardon, Alexander Solyom, Thierry Levade, Edward H. Schuchman, Steffany A. L. Bennett, David A. Dyment, Toni S. Pearson   +16 more
wiley   +1 more source

A transient inflammatory response contributes to oxaliplatin neurotoxicity in mice

Annals of Clinical and Translational Neurology, Volume 9, Issue 12, Page 1985-1998, December 2022., 2022
Abstract Objectives Peripheral neuropathy is a relevant dose‐limiting adverse event that can affect up to 90% of oncologic patients with colorectal cancer receiving oxaliplatin treatment. The severity of neurotoxicity often leads to dose reduction or even premature cessation of chemotherapy.
Aina Calls, Abel Torres‐Espin, Marc Tormo, Laura Martínez‐Escardó, Núria Bonet, Ferran Casals, Xavier Navarro, Víctor J. Yuste, Esther Udina, Jordi Bruna   +9 more
wiley   +1 more source

Making tau amyloid models in vitro: a crucial and underestimated challenge

FEBS Letters, EarlyView.
This review highlights the challenges of producing in vitro amyloid assemblies of the tau protein. We review how accurately the existing protocols mimic tau deposits found in the brain of patients affected with tauopathies. We discuss the important properties that should be considered when forming amyloids and the benchmarks that should be used to ...
Julien Broc, Clara Piersson, Yann Fichou
wiley   +1 more source

Unraveling Mycobacterium tuberculosis acid resistance and pH homeostasis mechanisms

FEBS Letters, EarlyView.
Mycobacterium tuberculosis exhibits a remarkable resilience to acid stress. In this Review, we discuss some of the molecular mechanisms and metabolic pathways used by the tubercle bacilli to adapt and resist host‐mediated acid stress. Mycobacterium tuberculosis (Mtb) is a successful pathogen that has developed a variety of strategies to survive and ...
Janïs Laudouze, Stéphane Canaan, Alexandre Gouzy, Pierre Santucci   +3 more
wiley   +1 more source

The solution supramolecular structure of α2 → 8 polysialic acid suggests a structural cause for its low immunogenicity

FEBS Letters, EarlyView.
α2 → 8 polysialic acid elicits poor immunogenicity. Small‐angle scattering shows a supramolecular structure with parallel‐chain binding, although in different forms at μm and mm calcium. The major histocompatibility complex requires molecular weights around 2000 Da to produce antibodies, and 2000 Da polysialic oligomers will bind in these structures ...
Kenneth A. Rubinson
wiley   +1 more source

Refining the NaV1.7 pharmacophore of a class of venom‐derived peptide inhibitors via a combination of in silico screening and rational engineering

FEBS Letters, EarlyView.
Venom peptides have shown promise in treating pain. Our study uses computer screening to identify a peptide that targets a sodium channel (NaV1.7) linked to chronic pain. We produced the peptide in the laboratory and refined its design, advancing the search for innovative pain therapies.
Gagan Sharma, Jennifer R. Deuis, Xinying Jia, Theo Crawford, Sassan Rahnama, Eivind A. B. Undheim, Irina Vetter, Yanni K. ‐Y. Chin, Mehdi Mobli   +8 more
wiley   +1 more source

Subpar reporting of pre‐analytical variables in RNA‐focused blood plasma studies

Molecular Oncology, EarlyView.
Pre‐analytical variables strongly influence the analysis of extracellular RNA (cell‐free RNA; exRNA) derived from blood plasma. Their reporting is essential to allow interpretation and replication of results. By evaluating 200 exRNA studies, we pinpoint a lack of reporting pre‐analytical variables associated with blood collection, plasma preparation ...
Céleste Van Der Schueren, Philippe Decruyenaere, Francisco Avila Cobos, Johanna Bult, Jill Deleu, Laudonia Lidia Dipalo, Hetty Hilde Helsmoortel, Eva Hulstaert, Annelien Morlion, Elena Ramos Varas, Kathleen Schoofs, Wim Trypsteen, Eveline Vanden Eynde, Hanne Van Droogenbroeck, Kimberly Verniers, Jo Vandesompele, Anneleen Decock   +16 more
wiley   +1 more source

Cellular liquid biopsy provides unique chances for disease monitoring, preclinical model generation and therapy adjustment in rare salivary gland cancer patients

Molecular Oncology, EarlyView.
We quantified and cultured circulating tumor cells (CTCs) of 62 patients with various cancer types and generated CTC‐derived tumoroid models from two salivary gland cancer patients. Cellular liquid biopsy‐derived information enabled molecular genetic assessment of systemic disease heterogeneity and functional testing for therapy selection in both ...
Nataša Stojanović Gužvić, Florian Lüke, Steffi Treitschke, Andrea Coluccio, Martin Hoffmann, Giancarlo Feliciello, Adithi Ravikumar Varadarajan, Xin Lu, Kathrin Weidele, Catherine Botteron, Silvia Materna–Reichelt, Felix Keil, Katja Evert, Florian Weber, Thomas Schamberger, Michael Althammer, Jirka Grosse, Dirk Hellwig, Christian Schulz, Stephan Seitz, Peter Ugocsai, Anke Schlenska‐Lange, Roman Mayr, Ulrich Kaiser, Wolfgang Dietmaier, Bernhard Polzer, Jens Warfsmann, Kamran Honarnejad, Tobias Pukrop, Daniel Heudobler, Christoph A. Klein, Christian Werno   +31 more
wiley   +1 more source

Combined spatially resolved metabolomics and spatial transcriptomics reveal the mechanism of RACK1‐mediated fatty acid synthesis

Molecular Oncology, EarlyView.
The authors analyzed the spatial distributions of gene and metabolite profiles in cervical cancer through spatial transcriptomic and spatially resolved metabolomic techniques. Pivotal genes and metabolites within these cases were then identified and validated.
Lixiu Xu, Jinqiu Li, Junqi Ma, Ayshamgul Hasim   +3 more
wiley   +1 more source

Integrative systems‐level analysis reveals a contextual crosstalk between hypoxia and global metabolism in human breast tumors

Molecular Oncology, EarlyView.
Breast tumor samples scored for metabolic deregulation (M1 to M3) were given a hypoxia score (HS). The highest HS occurred in patients with strongest metabolic deregulation (M3), supporting tumor aggressiveness. HS correlated with the highest number of metabolic pathways in M1. This suggests hypoxia to be an early event in metabolic deregulation.
Raefa Abou Khouzam, Salem Chouaib, Mohammad Askandar Iqbal   +2 more
wiley   +1 more source