| Literature DB >> 33754349 |
Suella Martino1,2,3, Jean-Benoit Arlet3,4, Marie-Hélène Odièvre1,2,3,5, Vincent Jullien4, Martina Moras1,2,3, Claude Hattab1,2,3, Thibaud Lefebvre3,6,7, Laurent Gouya3,6,7, Mariano A Ostuni1,2,3, Sophie D Lefevre1,2,3, Caroline Le Van Kim1,2,3.
Abstract
Sickle cell disease (SCD) is characterised by chronic haemolysis and oxidative stress. Herein, we investigated 30 SCD patients and found 40% with elevated mitochondria levels (SS-mito+ ) in their mature red blood cells, while 60% exhibit similar mitochondria levels compared to the AA group (SS-mito- ). The SS-mito+ patients are characterised by higher reticulocytosis and total bilirubin levels, lower foetal haemoglobin, and non-functional mitochondria. Interestingly, we demonstrated decreased levels of mitophagy inducers, PINK1 and NIX, and higher levels of HSP90 chaperone in their red cells. Our results highlighted for the first time an abnormal retention of mitochondria in SCD linked with mitophagy-related proteins.Entities:
Keywords: PINK1/NIX; mitochondria; mitophagy; sickle cell disease
Year: 2021 PMID： 33754349 DOI： 10.1111/bjh.17416
Source DB: PubMed Journal: Br J Haematol ISSN： 0007-1048 Impact factor: 6.998