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Burton, E.

Publications and source records attributed to Burton, E..

11 recordsLinked to original sources

The HUSH Complex Dictates EBV-transformed B cell Sensitivity to NK Cell Surveillance Through Repression of NKG2A Ligand γ-Proto-cadherin

Natural Killer (NK) cells control Epstein-Barr virus (EBV), though how EBV+ B-cells escape NK surveillance to form tumors remains unknown. To gain insights, we performed a human genome-wide CRISPR-Cas9 screen in EBV-transformed lymphoblastoid cell lines (LCL). This revealed that the HUSH complex maintains LCL sensitivity to NK. HUSH knockout (KO) de-repressed LCL protocadherin gamma (PCDHG), typically expressed by neurons. PCDHG expression protected LCLs from NK and was necessary for HUSH KO-driven NK resistance. CRISPR analyses revealed NKG2A/CD94 as the PCDHG NK inhibitory counter-receptor, and NKG2A KO restored NK lysis of HUSH KO LCLs. HUSH perturbation impaired NK control of murine LCL xenografts in vivo. A subset of follicular lymphoma (FL) upregulate PCDHG, and PCDHG KO enhanced FL lysis by NK. Therefore, HUSH plays dual roles in foreign DNA surveillance and in support of NK, potentially as a neuronal dont kill me signal that can be exploited by transformed cells.

immunology↗

AN UPDATED POPULATION ESTIMATE FOR NORTHERN GANNETS ACROSS THEIR NORTH-EAST ATLANTIC BREEDING RANGE FOLLOWING THE 2022 OUTBREAK OF HIGH PATHOGENICITY AVIAN INFLUENZA

Northern gannets (Morus bassanus) have been regarded as a seabird success story, due to population increases throughout the 20th and 21st century contrasting with global seabird declines. However, in 2022 gannets experienced a severe outbreak of High Pathogenicity Avian Influenza (HPAI) across their global distribution, leading to an urgent need to reassess their population status. This study presents breeding gannet census numbers for 2023/24 from all colonies across the North-East Atlantic metapopulation (Great Britain, Ireland, the Channel Islands, Iceland, Norway, the Faroe Islands, France, Germany, Russia). Gannet numbers decreased by 17% across the North-East Atlantic metapopulation between the 2013/14 and 2023/24 census from 414,598 to 345,854 apparently occupied sites (AOS), a global decrease of at least 13%. The bulk of the reduction in AOS was driven by the largest colonies (>10,000 AOS) each losing tens of thousands of AOS. These figures likely underestimate the impact of the HPAI outbreak worldwide, since most colonies will have increased between the last census in 2013/14 and the 2022 HPAI outbreak, and the Canadian breeding population was last counted pre-HPAI outbreak. Scotland still holds the largest proportion of both the North-East Atlantic metapopulation (59%), and the world population (46%), while Great Britain, Ireland and the Channel Islands together hold 83% of the North-East Atlantic metapopulation and 64% of the world population. This study not only presents an updated population census for gannets in the North-East Atlantic but illustrates the large-scale impacts of a disease outbreak on a seabird species across its global range and highlights the importance of more regular census efforts to better quantify the demographic consequences of such events.

ecology↗

CRISPR Screens Reveal Epstein-Barr Virus-activated JunB as a Key Lymphoblastoid B cell Dependency Factor that Represses Cyclin Dependent Kinase Inhibitor P18INK4c

Epstein-Barr virus (EBV) persistently infects over 95% of adults worldwide and is associated with a range of cancers, including lymphomas and epithelial malignancies. Despite advances in understanding EBV biology, targeted therapies for EBV-associated cancers remain limited. To identify novel dependencies in EBV-infected cancers, we performed genome-wide CRISPR-Cas9 loss-of-function screens in EBV+ lymphoblastoid versus Burkitt lymphoma cells, which differ by EBV latency programs. JunB emerged as a critical LCL-selective host dependency factor. LCL JunB knockout significantly decreased proliferation, with reduced G2/M progression, but without inducing apoptosis. JunB was more highly expressed in B cells with the EBV latency III than latency I program and correlated with LMPist1 levels in newly infected B cells. LMP1 stimulated JunB expression in a manner dependent on its cytoplasmic tail TES1/CTAR1 region and on canonical NF-{kappa}B. EBV-activated JunB played an obligatory role in repression of the G1/S phase inhibitor CDKN2C/p18INK4c in LCLs but not Burkitt B cells. These findings establish an LMP1-JunB-p18INK4c axis as essential for EBV-driven lymphoblastoid B cell proliferation, suggest JunB-mediated cross-talk between Epstein-Barr nuclear antigens and LMP1, and highlight JunB as a potential therapeutic target for EBV-associated lymphoproliferative disorders.

microbiology↗

Arginine Metabolism Supports De Novo Pyrimidine Biosynthesis to Block DNA Damage and Maintain Epstein-Barr Virus Latency

Incompletely understood mechanisms serve to maintain Epstein-Barr virus (EBV) latency in most B-cell states, in which viral oncogene(s) are expressed but lytic antigens are repressed. Shortly after EBVs discovery and even before it was named, early pioneers Werne and Gertrude Henle identified that restriction of extracellular arginine de-represses EBV lytic antigens within Burkitt lymphoma tumor cells. However, for nearly 60 years, it has remained unknown how arginine metabolism supports EBV latency. To gain insights, we performed an amino acid restriction screen in Burkitt cell lines. This confirmed that arginine restriction was sufficient to trigger EBV reactivation in Burkitt B-cells and gastric carcinoma models. Arginine restriction strongly impaired de novo pyrimidine biosynthesis, and CRISPR or chemical genetic blockade of pyrimidine biosynthesis enzymes induced EBV immediate early and early lytic gene expression. However, arginine restriction blocked EBV lytic DNA replication and consequently also late gene expression, suggesting an abortive lytic cycle. Arginine restriction triggered DNA damage, which was an important driver of arginine restriction-driven EBV reactivation. Arginine restriction and DNA hypomethylation synergistically increased EBV reactivation. Together, our results highlight arginine and pyrimidine metabolism as potential targets for EBV lytic antigen induction therapy in B and epithelial cell contexts. ImportanceAltered metabolism is a hallmark of cancer, frequently increasing transformed cell dependence on extracellular amino acid supply. Despite current interest in EBV lytic antigen induction therapy, in which viral lytic reactivation sensitizes tumors to the highly cytotoxic effects of the antiviral ganciclovir, there has been no systemic study of extracellular amino acid that controls EBV latency. We identified that arginine uptake was important for the maintenance of EBV latency in both Burkitt lymphoma and gastric carcinoma contexts. Metabolic pathway analyses highlighted that arginine uptake and metabolism was required to supply pyrimidines. Disruption of arginine metabolism or de novo pyrimidine synthesis caused DNA damage. As arginine restriction was also found to cause Burkitt DNA hypermethylation, we provide evidence that the combination of arginine restriction and DNA hypomethylation by decitabine or by CRISPR approaches together induced EBV reactivation more highly than either alone, suggesting a therapeutic approach.

microbiology↗

The Pesticide Chlorpyrifos Increases the Risk of Parkinson's Disease

Background and PurposePesticides have been associated with an increased risk of Parkinsons disease (PD), but it is unclear which specific pesticides contribute to this association and whether it is causal. Since chlorpyrifos (CPF) exposure has been implicated as a risk factor for PD, we investigated its association to incident PD and if this association is biologically plausible using human, rodent, and zebrafish (ZF) studies. MethodsThe association of CPF with PD was assessed using the UCLA PEG study (829 PD and 824 control subjects), and proximity-based exposure estimates from living or working near agricultural CPF use. For the mammalian studies, 6 months old male C57BL/6 mice were divided into two groups, CPF and controls, for open field, rotarod, and wire hang behavioral testing. Mice were then exposed to CPF in an inhalation chamber (0.65-2.9 mg/m3/day) for 6 hrs./day 5 days/wk., whereas control mice were exposed to vehicle alone. Behavioral tests were performed before and 2.5 months after CPF exposure following a 3-day washout. Mice were then perfused for immunohistochemical analysis. For the mechanistic studies, ZF embryos were treated with CPF (250 nM) 24 hours post fertilization for 5-7 days. Behavioral testing was performed using the Viewpoint Imaging System. Neuronal loss and microglial activation were determined using immunohistochemistry. Neuronal autophagic flux was determined using autophagy modulators in GFP-LC3 transgenic ZF and Western blots. ResultsLong-term residential CPF exposure was linked to an increased risk of developing PD with an odds ratio of 2.68 (CI 1.58-4.55). Mice exposed to aerosolized CPF developed motor impairment and a significant loss of dopaminergic neurons in the substantia nigra and activation of microglia. TH positive neurons in the substantia nigra (SN) had significantly higher levels of phosphoserine 129 (pS129) -synuclein (-syn), a marker for pathological phosphorylated -syn, and ubiquitin. In contrast, neither pS129 -syn or ubiquitin accumulated in TH neurons in the VTA after CPF exposure. Consistent with the mice data, CPF exposure resulted in impairment of locomotor activity and selective loss of aminergic neurons in ZF. We also found an increase in neuronal apoptosis and microglial activation. Importantly, dopamine neuron loss was found to be at least partially dependent on {gamma}1-synuclein (closest functional homologue to human -syn) as neuronal loss did not occur in {gamma}1-synuclein knockout ZF. Using an in vivo ZF assay, we found impaired autophagic flux and an increase in lysosomal labelling within the zebrafish brain. CPF exposure also led to elevated {gamma}1-synuclein and p62 (autophagic cargo protein) levels consistent with impaired degradation. Furthermore, induction of autophagy was protective, supporting the hypothesis that impaired autophagic flux is at least partially responsible for neuron loss following CPF exposure. ConclusionsCPF exposure is associated with an increased risk of developing PD and this association is likely causal since PD-like pathology was recapitulated in animal models. Furthermore, impaired autophagic flux appears to underly this toxicity, a pathway implicated in the pathogenesis of PD.

neuroscience↗

Epstein-Barr Virus Latent Membrane Protein 1 Subverts IMPDH pathways to drive B-cell oncometabolism

Epstein-Barr virus (EBV) is associated with multiple types of cancers, many of which express the key viral oncoprotein Latent Membrane Protein 1 (LMP1). LMP1 is the only EBV-encoded protein whose expression is sufficient to transform both epithelial and B-cells. Although metabolism reprogramming is a cancer hallmark, much remains to be learned about how LMP1 alters lymphocyte oncometabolism. To gain insights into key B-cell metabolic pathways subverted by LMP1, we performed systematic metabolomic analyses on B cells with conditional LMP1 expression. This approach highlighted that LMP highly induces de novo purine biosynthesis, with xanthosine-5-P (XMP) as one of the most highly LMP1-upregulated metabolites. Consequently, IMPDH inhibition by mycophenolic acid (MPA) triggered apoptosis of LMP1-expressing EBV-transformed lymphoblastoid cell lines (LCL), a key model for EBV-driven immunoblastic lymphomas. Whereas MPA instead caused growth arrest of Burkitt lymphoma cells with the EBV latency I program, conditional LMP1 expression triggered their apoptosis. Although both IMPDH isozymes are expressed in LCLs, only IMPDH2 was critical for LCL survival, whereas both contributed to proliferation of Burkitt cells with the EBV latency I program. Both LMP1 C-terminal cytoplasmic tail domains critical for primary human B-cell transformation were important for XMP production, and each contributed to LMP1-driven Burkitt cell sensitivity to MPA. MPA also de-repressed EBV lytic antigens including LMP1 in latency I Burkitt cells, highlighting crosstalk between the purine biosynthesis pathway and the EBV epigenome. These results suggest novel oncometabolism-based therapeutic approaches to LMP1-driven lymphomas. IMPORTANCEAltered metabolism is a hallmark of cancer, yet much remains to be learned about how EBV rewires host cell metabolism to support multiple malignancies. While the oncogene LMP1 is the only EBV-encoded gene that is sufficient to transform murine B-cells and rodent fibroblasts, knowledge has remained incomplete about how LMP1 alters host cell oncometabolism to aberrantly drive infected B-cell growth and survival. Likewise, it has remained unknown whether LMP1 expression creates metabolic vulnerabilities that can be targeted by small molecule approaches to trigger EBV-transformed B-cell programmed cell death. We therefore used metabolomic profiling to define how LMP1 signaling remodels the B-cell metabolome. We found that LMP1 upregulated purine nucleotide biosynthesis, likely to meet increased demand. Consequently, LMP1 expression sensitized Burkitt B-cells to growth arrest upon inosine monophosphate dehydrogenase blockade. Thus, while LMP1 itself may not be a therapeutic target, its signaling induces dependence on downstream druggable host cell nucleotide metabolism enzymes, suggesting rational therapeutic approaches.

microbiology↗

Imipridones inhibit tumor growth and improve survival in an orthotopic liver metastasis mouse model of human uveal melanoma

PurposeUveal melanoma (UM) is a highly aggressive disease with very few treatment options. We previously demonstrated that mUM is characterized by high oxidative phosphorylation (OXPHOS). Here we tested the anti-tumor, signaling and metabolic effects of imipridones, CLPP activators which reduce OXPHOS indirectly and have demonstrated safety in patients. Experimental DesignWe assessed CLPP expression in UM patient samples. We tested the effects of imipridones (ONC201, ONC212) on the growth, survival, signaling and metabolism of UM cell lines in vitro, and for therapeutic effects in vivo in UM liver metastasis models. ResultsCLPP expression was confirmed in primary and mUM patient samples. ONC201/212 treatment of UM cell lines in vitro decreased OXPHOS effectors, inhibited cell growth and migration, and induced apoptosis. ONC212 increased metabolic stress and apoptotic pathways, inhibited amino acid metabolism, and induced cell death-related lipids. ONC212 also decreased tumor burden and increased survival in vivo in two UM liver metastasis models. ConclusionImipridones are a promising strategy for further testing and development in mUM.

cancer biology↗

Characterization of Target Gene Regulation by the Two Epstein-Barr Virus Oncogene LMP1 Domains Essential for B-cell Transformation

The Epstein-Barr virus (EBV) oncogene latent membrane protein 1 (LMP1) mimics CD40 signaling and is expressed by multiple malignancies. Two LMP1 C-terminal cytoplasmic tail regions, termed transformation essential sites (TES) 1 and 2, are critical for EBV transformation of B lymphocytes into immortalized lymphoblastoid cell lines (LCL). However, TES1 versus TES2 B-cell target genes have remained incompletely characterized, and whether both are required for LCL survival has remained unknown. To define LCL LMP1 target genes, we profiled transcriptome-wide effects of acute LMP1 CRISPR knockout (KO) prior to cell death. To then characterize specific LCL TES1 and TES2 roles, we conditionally expressed wildtype, TES1 null, TES2 null or double TES1/TES2 null LMP1 alleles upon endogenous LMP1 KO. Unexpectedly, TES1 but not TES2 signaling was critical for LCL survival. The LCL dependency factor cFLIP, which plays obligatory roles in blockade of LCL apoptosis, was highly downmodulated by loss of TES1 signaling. To further characterize TES1 vs TES2 roles, we conditionally expressed wildtype, TES1 and/or TES2 null LMP1 alleles in two Burkitt models. Systematic RNAseq analyses revealed gene clusters that responded more strongly to TES1 versus TES2, that respond strongly to both or that are oppositely regulated. Robust TES1 effects on cFLIP induction were again noted. TES1 and 2 effects on expression of additional LCL dependency factors, including BATF and IRF4, and on EBV super-enhancers were identified. Collectively, these studies suggest a model by which LMP1 TES1 and TES2 jointly remodel the B-cell transcriptome and highlight TES1 as a key therapeutic target. ImportanceEpstein-Barr virus (EBV) causes multiple human cancers, including B-cell lymphomas. In cell culture, EBV converts healthy human B-cells into immortalized ones that grow continuously, which model post-transplant lymphomas. Constitutive signaling from two cytoplasmic tail domains of the EBV oncogene Latent Membrane Protein 1 (LMP1) is required for this transformation, yet there has not been systematic analysis of their host gene targets. We identified that only signaling from the membrane proximal domain is required for survival of these EBV-immortalized cells and that its loss triggers apoptosis. We identified key LMP1 target genes, whose abundance changed significantly with loss of LMP1 signals, or that were instead upregulated in response to switching on signaling by one or both LMP1 domains in an EBV-uninfected human B-cell model. These included major anti-apoptotic factors necessary for EBV-infected B-cell survival. Bioinformatics analyses identified clusters of B-cell genes that respond differently to signaling by either or both domains.

cancer biology↗

Enhancer Reprogramming in Melanoma Immune Checkpoint Therapy Resistance

Immune checkpoint blockade (ICB) therapy has improved long-term survival for patients with advanced melanoma. However, there is critical need to identify potential biomarkers of response and actionable strategies to improve response rates. Through generation and analysis of 148 chromatin modification maps for 36 melanoma samples from patients treated with anti-PD- 1, we identified significant enrichment of active enhancer states in non-responders at baseline. Analysis of an independent cohort of 20 samples identified a set of 437 enhancers that predicted response to anti-PD-1 therapy (Area Under the Curve of 0.8417). The activated non-responder enhancers marked a group of key regulators of several pathways in melanoma cells (including c- MET, TGF{beta}, EMT and AKT) that are known to mediate resistance to ICB therapy and several checkpoint receptors in T cells. Epigenetic editing experiments implicated involvement of c-MET enhancers in the modulation of immune response. Finally, inhibition of enhancers and repression of these pathways using bromodomain inhibitors along with anti-PD-1 therapy significantly decreased melanoma tumor burden and increased T-cell infiltration. Together, these findings identify a potential enhancer-based biomarker of resistance to anti-PD-1 and suggest enhancer blockade in combination with ICB as a potential strategy to improve responses.

cancer biology↗

Characterization Of Yellow Root Cassava And Food Products: Investigation Of Cyanogenic Glycosides And Pro-Vitamin A

ObjectiveCyanide is a highly toxic compound, and the consumption of products containing cyanide is of singificant public health concern. In contrast, {beta}-carotene possesses essential nutritional attributes related to human health, therefore the characterisation and quanfication of both compounds in food products is both fundamental and necessary. This investigation sought to identify the cyanide and {beta}-carotene levels in two flours produced from the roots of two varieties of cassava (Manihot esculenta crantz), namely UMUCASS-38 (TMS 01/1371) and NR 8082, and their associated food products. ResultsThe fresh tuber, raw flour and food products were analysed for levels of residual cyanide and {beta}-carotene using standard analytical methods. The cyanide content of NR 8082 (18.01{+/-}0.01 ppm) and UMUCASS 38 (17.02{+/-}0.02 ppm) flours were significantly higher (p < 0.05) than the residual cyanide levels determined in the cookies (10.00{+/-}0.00 ppm) and cake (7.10{+/-}0.14 ppm). The levels of {beta}-carotene determined in the sample varied significantly (p < 0.05). The highest levels of {beta}-carotene (6.53{+/-}0.02 {micro}g/g) were determined in raw roots of UMUCASS 38 while NR 8082 levels of {beta}-carotene were 1.12{+/-}0.02 {micro}g/g. Processing the roots into flour reduced the {beta}-carotene content to 4.78{+/-}0.01 {micro}g/g and 0.76{+/-}0.02 {micro}g/g in UMUCASS 38 and NR8082 flours, respectively. Cookies and cake produced from flour derived from the UMUCASS 38 variety had 2.15{+/-}0.01 {micro}g/g and 2.84{+/-}0.04 {micro}g/g of {beta}-carotene, respectively.

biochemistry↗

Regeneration of the zebrafish retinal pigment epithelium after widespread genetic ablation

The retinal pigment epithelium (RPE) is a specialized monolayer of pigmented cells within the eye that is critical for maintaining visual system function. Diseases affecting the RPE have dire consequences for vision, and the most prevalent of these is atrophic (dry) age-related macular degeneration (AMD), which is thought to result from RPE dysfunction and degeneration. An intriguing possibility for treating RPE degenerative diseases like atrophic AMD is the stimulation of endogenous RPE regeneration; however, very little is known about the mechanisms driving successful RPE regeneration in vivo. Here, we developed a zebrafish transgenic model (rpe65a:nfsB-GFP) that enabled ablation of large swathes of mature RPE. RPE ablation resulted in rapid RPE degeneration, as well as degeneration of Bruchs membrane and underlying photoreceptors. Using this model, we demonstrate for the first time that larval and adult zebrafish are capable of regenerating a functional RPE monolayer after RPE ablation. Regenerated RPE cells first appear at the periphery of the RPE, and regeneration proceeds in a peripheral-to-central fashion. RPE ablation elicits a robust proliferative response in the remaining RPE. Subsequently, proliferative cells move into the injury site and differentiate into RPE. BrdU pulse-chase analyses demonstrate that the regenerated RPE is likely derived from remaining peripheral RPE cells. Pharmacological inhibition of Wnt signaling significantly reduces cell proliferation in the RPE and delays overall RPE recovery. These data demonstrate that the zebrafish RPE possesses a robust capacity for regeneration and highlight a potential mechanism through which endogenous RPE regenerate in vivo.\n\nSIGNIFICANCE STATEMENTDiseases resulting in RPE degeneration are among the leading causes of blindness worldwide, and no therapy exists that can replace RPE or restore lost vision. One intriguing possibility is the development of therapies focused on stimulating endogenous RPE regeneration. For this to be possible, we must first gain a deeper understanding of the mechanisms underlying RPE regeneration. Here, we ablate mature RPE in zebrafish and demonstrate that zebrafish regenerate RPE after widespread injury. Injury-adjacent RPE proliferate and regenerate RPE, suggesting that they are the source of regenerated tissue. Finally, we demonstrate that Wnt signaling is required for RPE regeneration. These findings establish an in vivo model through which the molecular and cellular underpinnings of RPE regeneration can be further characterized.

developmental biology↗