期刊
BRITISH JOURNAL OF HAEMATOLOGY
卷 193, 期 5, 页码 988-993出版社
WILEY
DOI: 10.1111/bjh.17416
关键词
sickle cell disease; mitochondria; mitophagy; PINK1; NIX
类别
资金
- Region Ile de France
- European Union's Horizon 2020 research and innovation programme under Marie Sklodowska-Curie grant [665850]
- French National Research Agency [ANR-11-LABX-005]
This study revealed an abnormal retention of mitochondria in some SCD patients, along with specific biochemical changes. Meanwhile, alterations in levels of related proteins also play a role in the development of the disease.
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.
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