Model organism data evolving in support of translational medicine

DG Howe, JA Blake, YM Bradford, CJ Bult, BR Calvi… - Lab Animal, 2018 - nature.com
Abstract Model organism databases (MODs) have been collecting and integrating
biomedical research data for 30 years and were designed to meet specific needs of each …

A Drosophila model of neuronopathic Gaucher disease demonstrates lysosomal-autophagic defects and altered mTOR signalling and is functionally rescued by …

KJ Kinghorn, S Grönke, JI Castillo-Quan… - Journal of …, 2016 - Soc Neuroscience
Glucocerebrosidase (GBA1) mutations are associated with Gaucher disease (GD), an
autosomal recessive disorder caused by functional deficiency of glucocerebrosidase (GBA) …

Exploiting the Potential of Drosophila Models in Lysosomal Storage Disorders: Pathological Mechanisms and Drug Discovery

L Rigon, C De Filippis, B Napoli, R Tomanin, G Orso - Biomedicines, 2021 - mdpi.com
Lysosomal storage disorders (LSDs) represent a complex and heterogeneous group of rare
genetic diseases due to mutations in genes coding for lysosomal enzymes, membrane …

Into the deep: Exploring the molecular mechanisms of hyperactive behaviour induced by three rare earth elements in early life-stages of the deep-sea scavenging …

JA Riedel, I Smolina, C Donat, LH Svendheim… - Science of the Total …, 2024 - Elsevier
With increasing socio-economic importance of the rare earth elements and yttrium (REY),
Norway has laid out plans for REY mining, from land-based to deep-sea mining, thereby …

A ratiometric two-photon fluorescent probe for imaging hydrogen sulfide in lysosomes

W Feng, Z Mao, L Liu, Z Liu - Talanta, 2017 - Elsevier
Hydrogen sulfide (H 2 S) is a kind of gaseous signalling molecule that plays pivotal role in
various biological processes. So far, it is still a challenge to develop convenient and reliable …

A lysosome-targeted fluorescent chemodosimeter for monitoring endogenous and exogenous hydrogen sulfide by in vivo imaging

XJ Zou, YC Ma, LE Guo, WX Liu, MJ Liu… - Chemical …, 2014 - pubs.rsc.org
A lysosome-targeted fluorescent chemodosimeter, 1, was developed for monitoring
endogenous and exogenous H2S by in vivo imaging of HeLa cells, D. melanogaster and C …

Animal model contributions to congenital metabolic disease

CA Moro, W Hanna-Rose - Animal Models of Human Birth Defects, 2020 - Springer
Genetic model systems allow researchers to probe and decipher aspects of human disease,
and animal models of disease are frequently specifically engineered and have been …

Lipid metabolic perturbation is an early-onset phenotype in adult spinster mutants: a Drosophila model for lysosomal storage disorders

S Hebbar, A Khandelwal, R Jayashree… - Molecular Biology of …, 2017 - Am Soc Cell Biol
Intracellular accumulation of lipids and swollen dysfunctional lysosomes are linked to
several neurodegenerative diseases, including lysosomal storage disorders (LSD). Detailed …

A Great Catch for Investigating Inborn Errors of Metabolism—Insights Obtained from Zebrafish

M Breuer, SA Patten - Biomolecules, 2020 - mdpi.com
Inborn errors of metabolism cause abnormal synthesis, recycling, or breakdown of amino
acids, neurotransmitters, and other various metabolites. This aberrant homeostasis …

[HTML][HTML] Anthelmintic effects of a cationic toxin from a South American rattlesnake venom

C Dal Mas, JT Moreira, S Pinto, GG Monte, MB Nering… - Toxicon, 2016 - Elsevier
Despite the unquestionable importance of the highly cationic feature of several small
polypeptides with high content of positively charged amino acids for their biological …