Tandem P-selectin glycoprotein ligand immunoglobulin prevents lung vaso-occlusion in sickle cell disease mice.
Exp Hematol
; 84: 1-6.e1, 2020 04.
Article
in En
| MEDLINE
| ID: mdl-32243995
ABSTRACT
Sickle cell disease (SCD) is a monogenic disorder estimated to affect more than three million people worldwide. Acute systemic painful vaso-occlusive episode (VOE) is the primary reason for emergency medical care among SCD patients. VOE may also progress to acute chest syndrome (ACS), a type of acute lung injury and one of the primary reasons for mortality among SCD patients. Recently, P-selectin monoclonal antibodies were found to attenuate VOE in SCD patients and lung vaso-occlusion in transgenic humanized SCD mice, highlighting the therapeutic benefit of P-selectin inhibition in SCD. Here, we use quantitative fluorescence intravital lung microscopy (qFILM) to illustrate that tandem P-selectin-glycoprotein ligand-immunoglobulin (TSGL-Ig) fusion molecule containing four P-selectin binding sites, significantly attenuated intravenous (IV) oxyhemoglobin triggered lung vaso-occlusion in SCD mice. These findings highlight the therapeutic potential of TSGL-Ig in preventing VOE and ACS in SCD.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Vascular Diseases
/
Recombinant Fusion Proteins
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Immunoglobulins
/
P-Selectin
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Anemia, Sickle Cell
/
Lung Diseases
Limits:
Animals
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Female
/
Humans
/
Male
Language:
En
Journal:
Exp Hematol
Year:
2020
Document type:
Article
Affiliation country:
Panama