Search bioRxiv⌕ Search

Biology subjects

Picco, V.

Publications and source records attributed to Picco, V..

5 recordsLinked to original sources

MMRi62 induces iron depletion-driven apoptosis through a ferritin-independent mechanism

The modulation of iron metabolism is increasingly explored as a therapeutic strategy in cancer, particularly through the induction of ferroptosis. Given the central role of ferritin as a major intracellular iron buffer, targeting ferritin represents an attractive strategy to disrupt iron homeostasis; however, the lack of pharmacological approaches capable of selectively targeting ferritin currently limits the therapeutic exploitation of this vulnerability. MMRi62, initially developed to target the MDM2-MDM4 axis, has been proposed to induce ferroptosis via ferritin degradation. Here, we revisited this hypothesis in the context of medulloblastoma (MB). Contrary to this proposed mechanism, we found that MMRi62 does not trigger ferroptosis but instead induces robust apoptotic cell death. This effect is observed in both p53-mutant DAOY and p53 wild-type HD-MB03 cells, as well as in c-Myc/OTX2-driven medulloblastoma-like tumours genetically induced in brain organoids. Although ferritin degradation occurs upon treatment, genetic dissection demonstrates that neither ferritin itself nor ferritinophagy are required for MMRi62-induced cytotoxicity. Together, these findings rule out ferritin-dependent mechanisms as primary drivers of cytotoxicity. Instead, we uncovered that MMi62 induces a profound rewiring of iron metabolism consistent with a canonical iron starvation response, accompanied by a marked reduction in intracellular iron levels. Consistently, the UV-visible spectroscopic data were in agreement with the proposed iron-chelating properties of MMRi62. Taken together, our findings identify iron depletion-driven apoptosis, rather than ferroptosis, independently of ferritin degradation, as the primary mechanism of MMRi62 cytotoxicity in MB, refining its mode of action and highlighting iron homeostasis as a therapeutic vulnerability.

cancer biology↗

Deletion of Ferritin Heavy Chain Limits Tumor Growth and Promotes Iron-Dependent Stress in Medulloblastoma

Iron is essential for tumor proliferation and metabolic adaptation but becomes cytotoxic when unbuffered, creating a potential metabolic vulnerability. Ferritin, a conserved iron-storage complex, limits labile iron and establishes the upper threshold of iron tolerance in cancer cells. Here, we report the first ferritin heavy chain (FTH) knockout in a brain tumor model system. Although FTH loss was tolerated under basal conditions through adaptive remodeling of iron metabolism, it exposed profound vulnerabilities under iron stress. FTH deficiency lowered the threshold for iron toxicity, sensitizing medulloblastoma (MB) cells to both canonical ferroptosis and a mechanistically distinct iron-dependent cell death pathway. Oxidative iron stress impaired tumor growth and prolonged survival in orthotopic xenografts, whereas vitamin C-induced iron reduction triggered a selective, iron-dependent, but non-ferroptotic elimination of MB-like cells in tumor organoids. Notably, sensitivity to iron toxicity correlated strongly with cellular phenotype, with mesenchymal-like cells displaying greater susceptibility than epithelial-like counterparts. Collectively, these findings identify ferritin as a central regulator of iron tolerance in MB and establish iron toxicity, not via iron deprivation, as a therapeutically exploitable vulnerability. More broadly, this work provides a mechanistic framework for targeting iron metabolism through modulation of ferritin-dependent iron buffering and iron redox homeostasis in cancers. Graphical abstract O_FIG O_LINKSMALLFIG WIDTH=200 HEIGHT=170 SRC="FIGDIR/small/739635v1_ufig1.gif" ALT="Figure 1000"> View larger version (53K): org.highwire.dtl.DTLVardef@1c499a4org.highwire.dtl.DTLVardef@431c70org.highwire.dtl.DTLVardef@2b5borg.highwire.dtl.DTLVardef@11e9ac1_HPS_FORMAT_FIGEXP M_FIG C_FIG

cancer biology↗

Integrin-alpha V beta 3 is a fundamental factor in medulloblastoma tumorigenicity and radioresistance: A new game for an old player

Medulloblastoma (MB) is the most frequent solid tumor in children, localized in the brains posterior fossa. Its standard of care comprises maximal resection surgery followed by craniospinal irradiation and chemotherapy. Despite a long-term survival rate of 70%, wide disparities among patients have been observed. Relevant targets for naive and recurrent MB are urgently needed. Primary and recurrent MBs are characterized by aggressive invasion into surrounding brain tissue, active angiogenesis, and radioresistance. Integrin-v{beta}3 was a major driver of these features in glioblastoma. Nevertheless, such observations have not yet been reported in MB. Integrin-v{beta}3 was found to be expressed in a subset of MB patients. We investigated the role of integrin-v{beta}3 using MB-derived cell lines with {beta}3-subunit depletion or overexpression both in vitro and in vivo. Radioresistant MB cell lines were generated and showed increased integrin-v{beta}3 expression, which correlated with increased susceptibility to pharmacological integrin-v{beta}3 inhibition with cilengitide, a competitive ligand mimetic. Finally, we conducted single-photon emission computed tomography (SPECT)/magnetic resonance imaging (MRI) studies on orthotopic models using a radiolabeled integrin-v{beta}3 ligand (99mTc-RAFT-RGD). This approach offers the prospect of a novel predictive imaging modality in MB. Altogether, our data pave the way for SPECT/MRI-based selection of a subpopulation of MB patients eligible for integrin-v{beta}3-directed therapies. SIGNIFICANCEThis study demonstrates integrin-v{beta}3s fundamental role in MB tumorigenicity and radioresistance and the effect of its expression on cilengitide functional activity.

cancer biology↗

The antiangiogenic compound axitinib demonstrates low toxicity and anti-tumoral effects against medulloblastoma

Evolution of medulloblastoma (MB) treatments has increased the 5-year overall survival of to more than 70%. However, an increasing number of survivors face severe long-term adverse effects and associated morbidity due to multimodal treatments particularly harsh for the younger patients. Chemotherapeutic compounds inducing less adverse effects are key to improving the care of MB patients. The preclinical relevance of last generation anti-angiogenic compounds deserves to be fully assessed. Among these, axitinib showed the highest selectivity index for MB cells, efficiently reduced the growth rate of experimental tumors and led to less toxicity towards normal cells than did a reference treatment. In vivo, axitinib did not lead to acute toxicity in very young rats and was able to cross the blood brain barrier. Analysis of public databases shows that high expression of axitinib targets are of poor prognosis. Altogether, our results suggest that axitinib is a compelling candidate for MB treatment.

cancer biology↗

Pharmacological inhibition of the amino-acid transporter LAT1 demonstrates in vitro anti-neoplastic activity towards medulloblastoma

Most cases of medulloblastoma (MB) occur in young children. While the overall survival rate can be relatively high, current treatments combining surgery, chemo- and radiotherapy are very destructive for patient development and quality of life. Moreover, aggressive forms and recurrences of MB cannot be controlled by classical therapies. Therefore, new therapeutic approaches yielding good efficacy and low toxicity for healthy tissues are required to improve patient outcome. Cancer cells sustain their proliferation by optimizing their nutrient uptake capacities. The L-type amino acid transporter 1 (LAT1) is an essential amino acid carrier overexpressed in aggressive human cancers that was described as a potential therapeutic target. In this study, we investigated the therapeutic potential of JPH203, a LAT1-specific pharmacological inhibitor, on two independent MB cell lines belonging to subgroups 3 (HD-MB03) and Shh (DAOY). We show that while displaying low toxicity towards normal cerebral cells, JPH203 disrupts AA homeostasis, mTORC1 activity, proliferation and survival in MB cells. Moreover, we demonstrate that a long-term treatment with JPH203 does not lead to resistance in MB cells. Therefore, the present study suggests that targeting LAT1 with JPH203 is a promising therapeutic approach for MB treatment.

cancer biology↗