Pubblicazioni

Autori:

Nguyen, Kim-Anh; Matte, Alessandro; Foresti, Roberta; Federti, Enrica; Kiger, Laurent; Lefebvre, Cécile; Hocini, Hakim; Pelinski, Yanis; Kitagishi, Hiroaki; Bencheikh, Laura; Pirenne, France; DE FRANCESCHI, Lucia; Motterlini, Roberto; Bartolucci, Pablo

Titolo:

An Oral Carbon Monoxide-Releasing Molecule Protects against Acute Hyper-hemolysis in Sickle Cell Disease

Anno:

2024

Tipologia prodotto:

Articolo in Rivista

Tipologia ANVUR:

Articolo su rivista

Lingua:

Inglese

Referee:

No

Nome rivista:

Blood

ISSN Rivista:

0006-4971

Editore:

American Society of Hematology

Intervallo pagine:

1-14

Parole chiave:

Delay hemolysis transfusion reaction (DHTR), sickle cell disease (SCD), intravascular hemolysis, endothelial damage, transfusion, carbon monoxide (CO), CO-releasing molecules (CO-RMs)

Breve descrizione dei contenuti:

Acute hyper-hemolysis is a severe life-threatening complication in patients with sickle cell disease (SCD) that may occur during delayed hemolytic transfusion reaction (DHTR), or vaso-occlusive crises associated with multi-organ failure. Here, we developed in vitro and in vivo animal models to mimic endothelial damage during the early phase of hyper-hemolysis in SCD. We then used the carbon monoxide (CO)- releasing molecule CORM-401 and examined its effects against endothelial activation, damage, and inflammation inflicted by hemolysates containing red blood cell membrane-derived particles. The in vitro results revealed that CORM-401: 1) prevented the up-regulation of relevant pro-inflammatory, and pro-adhesion controlled by the nuclear factor kappa-light-chain-enhancer of activated B cells (NF- κB), and 2) abolished the expression of the nuclear factor erythroid-2-related factor 2 (Nrf2) that regulates the inducible antioxidant cell machinery. We also show in SCD mice that CORM-401 protects against hemolysate-induced acute damage of target organs such as the lung, liver, and kidney through modulation of NF-kB proinflammatory and Nrf2 antioxidant pathways. Our data demonstrate the efficacy of CORM-401 as a novel therapeutic agent to counteract hemolysate-induced organ damage during hyper-hemolysis in SCD. This approach might be considered as possible preventive treatment in high-risk situations such as SCD patients with history of DHTR.

Id prodotto:

139912

Handle IRIS:

11562/1127433

ultima modifica:

21 febbraio 2025

Citazione bibliografica:

Nguyen, Kim-Anh; Matte, Alessandro; Foresti, Roberta; Federti, Enrica; Kiger, Laurent; Lefebvre, Cécile; Hocini, Hakim; Pelinski, Yanis; Kitagishi, Hiroaki; Bencheikh, Laura; Pirenne, France; DE FRANCESCHI, Lucia; Motterlini, Roberto; Bartolucci, Pablo, An Oral Carbon Monoxide-Releasing Molecule Protects against Acute Hyper-hemolysis in Sickle Cell Disease «Blood» , 2024 , pp. 1-14

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