Search bioRxiv⌕ Search

Biology subjects

Hemphill, A.

Publications and source records attributed to Hemphill, A..

10 recordsLinked to original sources

A new semi-automated, motility-based screening assay for discovery of compounds with activity against the juvenile stage of Fasciola hepatica

Fasciola hepatica is a trematode parasite responsible for fasciolosis, a liver disease that affects humans and livestock worldwide. Together with other food-borne trematode infections, fasciolosis is considered a neglected tropical disease. Further, it imposes substantial agricultural losses due to infections in ruminants. No vaccine is currently available, and control heavily relies on drug treatment, especially with triclabendazole (TCBZ). However, the intensive use of TCBZ over the past four decades has led to increasing rates of treatment failures and the emergence of drug-resistant parasites. Therefore, the identification of new treatment options is an urgent priority. The currently available toolset for drug screening, however, is limited. To address this need, we established a novel, semi-automated, standardized, and objective screening assay based on motility monitoring of newly excysted juveniles using microscopic live imaging. The assay was validated by testing a panel of ten compounds with known anthelmintic properties, amongst them TCBZ (IC50: 1.5 {micro}M) and the new activator of the F. hepatica transient receptor potential melastatin (TRPM) ion channel, benzamidoquinazolinone (IC50: 1.05 {micro}M). In addition to these two compounds with known activity against F. hepatica, three compounds were identified as particularly promising with a fast onset of action and IC50 values in the nanomolar range: the salicylanilides MMV665807 (IC50: 44 nM), niclosamide (IC50: 32 nM), and its ethanolamine salt, niclosamide ethanolamine (IC50: 9 nM). Complementary live/dead staining revealed that only TCBZ displayed parasiticidal activity, while the other compounds, although leading to parasite paralysis, did not lead to parasite death within 72 hours. Scanning electron microscopy of drug treated parasites did not reveal any significant damage at concentrations corresponding to the IC50s, but strong phenotypes were visible at 20 {micro}M. The presented motility assay provides a robust method for the discovery of novel anthelmintic compounds and facilitates the ongoing effort to combat fasciolosis. Author SummaryFasciola hepatica, the common liver fluke, is a parasitic platyhelminth that infects the liver and biliary ducts of humans and livestock, causing fasciolosis, a Neglected Tropical Disease as defined by the World Health Organization. Triclabendazole is the drug of choice to treat humans and animals. However, its intensive use has led to the emergence of drug resistance resulting in treatment failures worldwide. The identification of novel drugs is therefore urgent. Here, we present a semi-automated and objective method to assess the activity of compounds on one of the key life stages of the parasite: the newly excysted juveniles (NEJ). This stage is highly motile and motility assessment can be exploited to screen for bioactive compounds. Using time-lapse imaging, we quantified NEJ movement after drug exposure. From a panel of ten tested reference anthelmintics, two known fasciolicides (triclabendazole and benzamidoquinazolinone) and three additional compounds (MMV665807, niclosamide, and niclosamide ethanolamine) displayed particularly strong activity and were selected for further investigation. This method represents a robust tool for drug screening and facilitates the discovery of new compounds against F. hepatica.

microbiology↗

Repurposing Niclosamide Ethanolamine for Alveolar Echinococcosis Reveals a Disconnect Between In Vitro Efficacy and In Vivo Outcome

BackgroundEchinococcosis is a zoonotic disease caused by cestodes of the genus Echinococcus. Alveolar echinococcosis (AE), caused by E. multilocularis, primarily affects the liver and shows infiltrative, tumor-like growth of the metacestode stage. If untreated, AE is lethal. AE remains a neglected disease with current treatments based on albendazole or mebendazole that are parasitostatic, and not curative, underscoring the need for more effective therapies. Niclosamide is a chlorinated salicylanilide derivative with proven activities against intestinal helminths but is inactive against tissue-dwelling helminths due to poor absorption and limited bioavailability. In this study, we repurposed niclosamide ethanolamine (NEN), a formulation with improved systemic exposure, for the treatment of E. multilocularis infection in vitro and in vivo. Methodology/Principal FindingsWe assessed the in vitro efficacy of niclosamide and NEN against E. multilocularis metacestode vesicles (IC50<0.2 {micro}M) and primary parasite cells (IC50<0.3 {micro}M), with active concentrations largely corresponding to NEN levels reachable in the liver. Metabolic analysis suggested that NEN acts as a mitochondrial uncoupler. Electron microscopy showed that NEN-treatments induced profound structural damage in the metacestode vesicle tissue, but mitochondrial ultrastructure was not notably affected. In mice intraperitoneally infected with E. multilocularis, NEN was orally administered during 9 weeks either alone, or in combination with albendazole. Pharmacokinetic analyses showed that NEN reached blood level concentrations above 1 {micro}M. However, the parasite burden in NEN-treated mice was not significantly reduced. Conclusions/SignificanceAlthough niclosamide and NEN demonstrated potent activity against E. multilocularis in vitro, this efficacy did not translate in the mouse model. The lack of in vivo activity could be attributed to several factors such as infection model, limited drug uptake by the parasite in the animal, or the rapid metabolization of the compound. Future studies should explore novel niclosamide derivatives and formulations to enhance efficacy against AE in vivo. Author SummaryAlveolar echinococcosis (AE) is a severe disease caused by the larval stage of the fox tapeworm Echinococcus multilocularis. The parasite forms tumor-like lesions in the liver and can spread to other organs. The currently licensed drugs for the treatment of AE are not always effective, require long-term use, and can cause side effects that frequently require treatment interruption. Therefore, safer and more efficacious treatment options are urgently needed. Niclosamide is frequently applied for the treatment of adult tapeworm infections in the intestine, but its limited uptake and low biodistribution renders the compound unsuitable for systemic treatment. In this study, we tested a non-toxic salt formulation, niclosamide ethanolamine (NEN), exhibiting improved absorption. In vitro, NEN was highly effective against E. multilocularis metacestode vesicles. It induced profound structural alterations in metacestode vesicles and impaired the mitochondrial membrane potential, and thus interfering in energy production. However, NEN was not effective against AE in experimentally infected mice. Our results suggest that NEN treatment appears promising in vitro, but to translate to the in vivo situation, new formulations and delivery strategies should be developed to increase absorption, bioavailability and metabolic stability of the compound for an effective treatment for AE.

pharmacology and toxicology↗

Bumped kinase inhibitor BKI-1708 interferes in cytokinesis and drives baryzoite stage conversion in the cyst-forming apicomplexan parasites Toxoplasma gondii, Neospora caninum and Besnoitia besnoiti

Bumped kinase inhibitors (BKIs) have demonstrated safety and promising efficacy against various apicomplexan pathogens both in vitro and in vivo. However, in the closely related cyst-forming coccidians T. gondii, Neospora caninum and Besnoitia besnoiti, in vitro treatments with a range of BKIs induced the conversion of intracellular tachyzoites into atypical multinucleated complexes (MNCs), also named "baryzoites". In this study, baryzoites of T. gondii, N. caninum and B. besnoiti generated through exposure of tachyzoites to 2.5 {micro}M BKI-1708 were comparatively assessed. TEM showed that baryzoites contained multiple nuclei, clustered together and separated from the cytoplasmic organelles of newly formed zoites. These zoites do not have outer tachyzoite plasma membrane, were unable to complete cytokinesis, remained intracellular, and were enclosed by a parasitophorous vacuole membrane. TEM demonstrated the presence of an electron-dense cyst wall-like components only in T. gondii baryzoites. Species-specific differences in antigen expression were observed by immunofluorescence using specific antibodies. Comparative proteomic analysis revealed consistent downregulation of ribosomal proteins, proteins associated with secretory organelles, as well as of transcription and translation factors in all baryzoites. Bradyzoite-specific markers were upregulated only in T. gondii baryzoites. In addition, common orthologues of two alveolin-domain filament proteins (IMC7 and IMC12) and a hypothetical protein (TGME49_236950, NCLIV_050850, BESB_060040) were detected at higher abundance in all treated parasites. Overall, baryzoites exhibit distinct phenotypic and proteomic profiles, with ambiguous expression of tachyzoite and bradyzoite antigens, and lacking complete cellular division under drug pressure, suggesting a reversible response to stress rather than progression into a fully differentiated form. SignificanceApicomplexan parasites cause serious diseases worldwide, yet treatment options remain limited. A promising group of drugs are BKIs. We investigated how BKI-1708 affects threclosely related T. gondii, N. caninum, and B. besnoiti. Instead of killing the parasites, the drug induced the formation of multinucleated structures termed "baryzoites". These baryzoites exhibited ambiguous characteristics during the actively growing and dormant stages of the parasite life cycle and were unable to complete normal cell division. Moreover, we observed other key similarities and differences among species including downregulation of ribosomal proteins and transcription/translation factors, while only T. gondii displayed cyst wall formation. Microscopy and proteomics demonstrated that baryzoites represent a distinct stage that is formed upon drug pressure and promotes parasite survival during prolonged drug exposure. These findings highlight the unexpected ways parasites adapt to drug treatment and provide new insights into how BKIs exert their activities.

systems biology↗

Midkine Attenuates Aβ Fibril Assembly and Amyloid Plaque Formation

Proteomic profiling of Alzheimers disease (AD) brains has identified numerous understudied proteins, including midkine (MDK), that are highly upregulated and correlated with A{beta} since the early disease stage, but their roles in disease progression are not fully understood. Here we present that MDK attenuates A{beta} assembly and influences amyloid formation in the 5xFAD amyloidosis mouse model. MDK protein mitigates fibril formation of both A{beta}40 and A{beta}42 peptides in Thioflavin T fluorescence assay, circular dichroism, negative stain electron microscopy, and NMR analysis. Knockout of Mdk gene in 5xFAD increases amyloid formation and microglial activation. Further comprehensive mass spectrometry-based profiling of whole proteome and detergent-insoluble proteome in these mouse models indicates significant accumulation of A{beta} and A{beta}-correlated proteins, along with microglial components. Thus, our structural and mouse model studies reveal a protective role of MDK in counteracting amyloid pathology in Alzheimers disease.

biochemistry↗

Preimaginal development of Aedes aegypti in brackish water produces adult mosquitoes with thicker cuticles and greater insecticide resistance

Aedes aegypti and Aedes albopictus mosquitoes, the principal vectors of many human arboviral diseases, lay eggs and undergo preimaginal development in fresh water habitats. They have also recently been found to develop in brackish water in coastal areas. Adult females emerging from brackish water-developing Ae. aegypti larvae are shown to possess thicker cuticles and greater resistance to common insecticides used against adults (adulticides) compared with fresh water Ae. aegypti. These findings are compatible with previous findings showing that brackish water Ae. aegypti larvae possess thicker cuticles and greater larvicide resistance. Greater resistance of salinity-tolerant Ae. aegypti to adulticides and larvicides is a hitherto unappreciated problem for controlling arboviral diseases and has implications also for other mosquito-borne diseases.

zoology↗

Establishment and application of unbiased in vitro drug screening assays for the identification of compounds against Echinococcus granulosus s.s.

Echinococcus multilocularis and E. granulosus s.l. are the causative agents of alveolar and cystic echinococcosis, respectively. Drug treatment options for these severe and neglected diseases are limited to benzimidazoles, which are not always efficacious, and adverse side effects are reported. Thus, novel and improved treatments are needed. In this study, the previously established platform for E. multilocularis in vitro drug assessment was adapted to E. granulosus s.s.. In a first step, in vitro culture protocols for E. granulosus s.s. were established. This resulted in the generation of large amounts of E. granulosus s.s. metacestode vesicles as well as germinal layer (GL) cells. In vitro culture of these cells formed metacestode vesicles displaying structural characteristics of metacestode vesicles generated in vivo. Next, drug susceptibilities of E. multilocularis and E. granulosus s.s. protoscoleces, metacestode vesicles and GL cells were comparatively assessed employing established assays including (i) metacestode vesicle damage marker release assay, (ii) metacestode vesicle viability assay, (iii) GL cell viability assay, and (iv) protoscolex motility assay. The standard drugs albendazole, buparvaquone, mefloquine, MMV665807, monepantel, niclosamide and nitazoxanide were included. MMV665807, niclosamide and nitazoxanide were active against the parasite in all four assays against both species. MMV665807 and monepantel were significantly more active against E. multilocularis metacestode vesicles, while albendazole and nitazoxanide were significantly more active against E. multilocularis GL cells. Albendazole displayed activity against E. multilocularis GL cells, but no effects were seen in albendazole-treated E. granulosus s.s. GL cells within five days. Treatment of protoscoleces with albendazole and monepantel had no impact on motility. Similar results were observed for both species with praziquantel and its enantiomers against protoscoleces. In conclusion, in vitro culture techniques and drug screening methods previously established for E. multilocularis were successfully implemented for E. granulosus s.s., allowing comparisons of drug efficacy between the two species.

pharmacology and toxicology↗

Single- and duplex TaqMan-quantitative PCR for determining the copy numbers of integrated selection markers during site-specific mutagenesis in Toxoplasma gondii by CRISPR-Cas9

Herein, we developed a single and a duplex TaqMan quantitative qPCR for absolute quantification of copy numbers of integrated dihydrofolate reductase-thymidylate synthase (mdhfr-ts) drug selectable marker for pyrimethamine resistance in Toxoplasma gondii knockouts (KOs). The single TaqMan qPCR amplifies a 174 bp DNA fragment of the inserted mdhfr-ts and of the wild-type (WT) dhfr-ts (wt-dhfr-ts) which is present as single copy gene in Toxoplasma and encodes a sensitive enzyme to pyrimethamine. Thus, the copy number of the dhfr-ts fragment in a given DNA quantity from KO parasites with a single site-specific integration should be twice the number of dhfr-ts copies recorded in the same DNA quantity from WT parasites. The duplex TaqMan qPCR allows simultaneous amplification of the 174 bp dhfr-ts fragment and the T. gondii 529-bp repeat element. Accordingly, for a WT DNA sample, the determined number of tachyzoites given by dhfr-ts amplification is equal to the number of tachyzoites determined by amplification of the Toxoplasma 529-bp, resulting thus in a ratio of 1. However, for a KO clone having a single site-specific integration of mdhfr-ts, the calculated ratio is 2. We then applied both approaches to test T. gondii RH mutants in which the major surface antigen (SAG1) was disrupted through insertion of mdhfr-ts using CRISPR-Cas9. Results from both assays were in correlation showing a high accuracy in detecting KOs with multiple integrated mdhfr-ts. Southern blot analyses using BsaBI and DraIII confirmed qPCRs results. Both TaqMan qPCRs are needed for reliable diagnostic of T. gondii KOs following CRISPR-Cas9-mediated mutagenesis, particularly with respect to off-target effects resulting from multiple insertions of mdhfr-ts. The principle of the duplex TaqMan qPCR is applicable for other selectable markers in Toxoplasma. TaqMan qPCR tools may contribute to more frequent use of WT Toxoplasma strains during functional genomics.

molecular biology↗

Comparative assessment of the effects of bumped kinase inhibitors on early zebrafish embryo development and pregnancy in mice.

Bumped kinase inhibitors (BKIs) are effective against a variety of apicomplexan parasites. Fifteen BKIs with promising in vitro efficacy against Neospora caninum tachyzoites, low cytotoxicity in mammalian cells, and no toxic effects in non-pregnant BALB/c mice, were assessed in pregnant mice. Drugs were emulsified in corn oil and applied by gavage for 5 days. Five BKIs did not affect pregnancy, 5 BKIs exhibited 15-35% of neonatal mortality, and 5 compounds caused strong effects (infertility, abortion, stillbirth and pup mortality). Additionally, the impact of these compounds on zebrafish (Danio rerio) embryo development was assessed by exposing freshly fertilized eggs to 0.2-50M of BKIs and microscopical monitoring of embryo development in a blinded manner during 4 days. We propose an algorithm that includes quantification of malformations and embryo deaths, and established a scoring system that allows to calculate an impact score (Si) that indicates at which concentrations BKIs visibly affect zebrafish embryo development. Comparison of the two models showed that for 9 compounds no clear correlation between Si and pregnancy outcome was visible. However, those 3 BKIs affecting zebrafish embryos only at high concentrations (40M or higher) did not impair mouse pregnancy at all, and those 3 compounds that inhibited zebrafish embryo development already at 0.2M showed detrimental effects in the pregnancy model. Thus, the zebrafish embryo development test has a limited predictive value to foresee pregnancy outcome in BKI-treated mice. We conclude, that maternal health-related factors such as cardiovascular, pharmacokinetic and/or bioavailability properties also contribute to BKI-pregnancy effects.

pathology↗

Depletion of cardiolipin induces major changes in energy metabolism in Trypanosoma brucei bloodstream forms

Cardiolipin (CL) is a mitochondrial inner membrane glycerophospholipid that associates with mitochondrial proteins to promote their activities and to facilitate protein complex and super-complex formation. Loss of CL leads to destabilized respiratory complexes and mitochondrial dysfunction. The role of CL in an organism lacking a conventional electron transport chain (ETC) has not been elucidated so far. We now report that in Trypanosoma brucei bloodstream forms, in which the ETC is truncated and composed of alternative oxidase and glycerol-3-phosphate dehydrogenase, and the mitochondrial membrane potential is generated by the hydrolytic action of the FoF1-ATP synthase, the inducible depletion of cardiolipin synthase (TbCls) is essential for parasite survival. Loss of TbCls and CL caused a rapid drop in ATP levels and a decline in the mitochondrial membrane potential. Unbiased proteomic analyses revealed a reduction in the levels of many mitochondrial proteins, most notably of FoF1-ATP synthase subunits and of the alternative oxidase, resulting in a strong decline of glycerol-3-phosphate-stimulated oxygen consumption. Interestingly, the changes in cellular respiration preceded the observed decrease in FoF1-ATPase stability, suggesting that the truncated ETC is the first pathway responding to the decline in CL. In addition, proteomic and metabolomic analyses revealed that select proteins and pathways involved in glucose and amino acid transport and metabolism are up-regulated during CL depletion, possibly as a stress response to restore cellular ATP levels.

biochemistry↗

Regulation of Hepatic MicroRNAs in Response to Early Stage Echinococcus multilocularis Egg Infection in C57BL/6 mice

In this study, we present a comprehensive analysis of the hepatic miRNA transcriptome in mice suffering from experimental primary alveolar echinococcosis (AE), a parasitic infection caused upon ingestion of Echinococcus multilocularis (E. multilocularis) eggs. At one month post-infection, infected C57BL/6 mice, along with non-infected control mice, were euthanized. Subsequently livers were collected and used for small RNA library preparation and next-generation sequencing (NGS). The most significantly dysregulated hepatic miRNAs were validated by Stem-loop RT-qPCR. We identified 28 miRNAs with significantly altered expression levels upon infection with E. multilocularis. Of these, 9 were up-regulated (fold change (FC) [&ge;] 1.5) and 19 were down-regulated (FC [&le;] 0.66) as compared to the non-infected controls. In infected liver tissues, mmu-miR-148a-3p and mmu-miR-101b-3p were 8- and 6-fold down-regulated, respectively, and the expression of mmu-miR-22-3p was reduced by 50%, compared to non-infected liver tissue. Conversely, significantly higher hepatic levels were noted for Mus musculus (mmu)-miR-21a-5p (FC = 2.3) and mmu-miR-122-5p (FC = 1.8). Down-regulated miRNAs were highly enriched in Reactome and KEGG pathways of angiogenesis and fatty acids biosynthesis. Moreover, relative mRNA expression levels of three pro-angiogenic (VEGFA, MTOR and HIF1-) and two lipogenic (FASN and ACSL1) genes were significantly higher in livers of E. multilocularis infected mice. Lastly, we studied the issue related to functionally mature arm selection preference (5p and/or 3p) from the miRNA precursor and showed that 9 pre-miRNAs exhibited different arm selection preferences in normal versus infected liver tissues. Our study provides first evidence of miRNA involvement in liver pathogenesis during AE. Our future research will focus on the characterization of miRNA transcriptome patterns in more advanced AE-stages towards the assessment of microRNA therapy for AE, and experimentally address functional characteristics of selected features presently found.\n\nAuthor SummaryVarious infectious diseases in humans have been associated with altered expression patterns of microRNAs (miRNAs), a class of small non-coding RNAs involved in negative regulation of gene expression. Herein, we revealed that significant alterations of miRNA expression occurred in murine liver subsequently to experimental infection with Echinococcus multilocularis (E. multilocularis) eggs when compared to non-infected controls. At the early stage of murine AE, hepatic miRNAs were mainly downregulated. Respective target genes of the most extensively downregulated miRNAs were involved in angiogenesis and fatty acid synthesis. Indeed, angiogenic and lipogenic genes were found to be significantly higher expressed in E. multilocularis infected livers relative to non-infected controls. These boosted cellular pathways are advantageous for development of the E. multilocularis metacestodes, since this larval stage is not able to undertake de novo fatty acid synthesis, and angiogenesis allows the larvae to be periparasitically supplied by oxygen and nutrients and to get rid of waste products. More research on the miRNA transcriptome at more advanced infection-stages, and on the role of angiogenesis in E. multilocularis larval growth and metastasis, is required to assess the usefulness of microRNA- and anti-angiogenic therapies against E. multilocularis infection.

microbiology↗