Abstract:
:In sickle cell disease, the aberrant assembly of hemoglobin fibers induces changes in red blood cell morphology and stiffness, which leads to downstream symptoms of the disease. Therefore, understanding of this assembly process will be important for the treatment of sickle cell disease. By performing the highest spatiotemporal resolution measurements (55 nm at 1 Hz) of single sickle hemoglobin fiber assembly to date and combining them with a model that accounts for the multistranded structure of the fibers, we show that the rates of sickle hemoglobin addition and loss have been underestimated in the literature by at least an order of magnitude. These results reveal that the sickle hemoglobin self-assembly process is very rapid and inefficient (4% efficient versus 96% efficient based on previous analyses), where net growth is the small difference between over a million addition-loss events occurring every second.
journal_name
Sci Advjournal_title
Science advancesauthors
Castle BT,Odde DJ,Wood DKdoi
10.1126/sciadv.aau1086subject
Has Abstractpub_date
2019-03-13 00:00:00pages
eaau1086issue
3issn
2375-2548pii
aau1086journal_volume
5pub_type
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