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Boido, M. M.

Publications and source records attributed to Boido, M. M..

2 recordsLinked to original sources

Challenges and promises in optimising a non-clinical protocol of intracerebroventricular human neural stem cell transplantation in ALS

Background and aimsNeural stem cell (NSC) transplantation holds promising therapeutic potential for neurodegenerative disorders like amyotrophic lateral sclerosis (ALS). However, pre-clinical studies and early-phase clinical trials have faced challenges hindering the effective clinical translation of this approach. Crucial hurdles include the side-effects of prolonged immunosuppression, concerns regarding cell origin and transplantation dosage, identification of the most appropriate therapeutic window, and invasiveness of surgical procedures. Here, we show challenges and promises in optimizing a non-clinical protocol to assess safety and efficacy of human NSC (hNSC) intracerebroventricular (ICV) transplantation for ALS. MethodsWe evaluated the safety of administering up to 1x106 hNSCs in immunodeficient mice and assessed their potential efficacy in reducing ALS hallmarks employing the SOD1G93A mouse model. Both, transient (15 days) and prolonged immunosuppression regimens, at low (15 mg/kg) and high (30 mg/kg) doses, were tested along with two different cell dosages (3x105 and 1x106). ResultsBilateral ICV injection of up to 1x106 hNSCs proved to be safe, with no evidence of tumor formation. At 40 days post-transplantation, hNSCs induced a trend toward delaying motor decline and reducing spinal cord (SC) microgliosis when transplanted under prolonged high-dose (30 mg/kg) immunosuppression. ConclusionsOur study suggests that: (i) a bilateral ICV transplantation of 1x106 hNSCs is safe and non-tumorigenic in immunodeficient hosts; (ii) sustained high-dose immunosuppression is essential for ensuring cell survival in immunocompetent mice; and (iii) hNSC transplantation may provide therapeutic benefits in ALS by delaying motor decline and reducing microgliosis. This study also highlights persisting hurdles that need to be further addressed, such as the aggressive murine immune response to exogenous cells.

neuroscience↗

BBB damage in aging causes brain iron deposits via astrocyte-neuron crosstalk and Hepc/Fpn1 pathway

During aging, iron accumulates in brains regions vulnerable to neurodegeneration: the cerebral cortex and the hippocampus. However, the mechanism of iron regulation in the brain remains scarce. Here, we demonstrated for the first time the involvement of the Hepcidin/Ferroportin1 pathway in brain iron metabolism during aging. We demonstrated the alteration of BBB integrity, that leads to increased iron permeability and deregulation of iron homeostasis during aging. We found that brain iron overload drives Hepcidin upregulation and, consequently, the inhibition of the iron exporter Ferroportin1, neuroinflammation and oxidative stress. Moreover, both in the cerebral cortex and hippocampus Ferroportin1 colocalizes with astrocytes, while the iron storage protein ferritin light-chain with neurons. This differential distribution suggests that astrocytes mediate iron shuttling and neurons are unable to metabolize it. Furthermore, we observed NCOA4-dependent ferritinophagy of ferritin heavy-chain isoforms determining the increase of light-chain enriched ferritin heteropolymers that are more efficient as iron chelators. Altogether, these data highlight the involvement of the Hepcidin/Ferroportin1 axis and NCOA4 during mice aging as a response to a higher iron influx to the brain.

neuroscience↗