The invisible string of coagulation, complement, iron, and inflammation in sickle cell disease

JD Beckman, EM Sparkenbaugh - Current opinion in hematology, 2023 - journals.lww.com
Several studies implicate dysregulation of the ADAMTS-13/VWF axis as playing a major role
in vaso-occlusive events (VOE) in SCD. Another highlight is reducing iron overload, which …

[HTML][HTML] Quantification of the zymogenicity and the substrate-induced activity enhancement of complement factor D

R Dani, G Oroszlán, R Martinusz, B Farkas… - Frontiers in …, 2023 - frontiersin.org
Complement factor D (FD) is a serine protease present predominantly in the active form in
circulation. It is synthesized as a zymogen (pro-FD), but it is continuously converted to FD by …

Complement involvement in sickle cell disease

MS Meuleman, LT Roumenina, A Grunenwald - La Presse Médicale, 2023 - Elsevier
Abstract Sickle Cell Disease (SCD) is a hereditary blood disorder characterized by the
presence of abnormal hemoglobin, leading to the formation of sickle-shaped red blood cells …

Development and characterization of narsoplimab, a selective MASP-2 inhibitor, for the treatment of lectin-pathway–mediated disorders

T Dudler, S Yaseen, WJ Cummings - Frontiers in Immunology, 2023 - frontiersin.org
Introduction Overactivation of the lectin pathway of complement plays a pathogenic role in a
broad range of immune-mediated and inflammatory disorders; mannan-binding lectin …

Factor XII contributes to thrombotic complications and vaso-occlusion in sickle cell disease

EM Sparkenbaugh, MW Henderson, M Miller-Awe… - Blood, 2023 - ashpublications.org
A hypercoagulable state, chronic inflammation, and increased risk of venous thrombosis and
stroke are prominent features in patients with sickle cell disease (SCD). Coagulation factor …

Inhibition of mannan-binding lectin associated serine protease (MASP)-2 reduces the cognitive deficits in a mouse model of severe traumatic brain injury

D Mercurio, F Pischiutta, S Seminara, F Tribuzio… - Journal of …, 2024 - Springer
The lectin pathway (LP) of complement mediates inflammatory processes linked to tissue
damage and loss of function following traumatic brain injury (TBI). LP activation triggers a …

Cold exposure induces vaso-occlusion and pain in sickle mice that depend on complement activation

ZK Ivy, JD Belcher, IA Khasabova, C Chen, JP Juliette… - Blood, 2023 - ashpublications.org
Vaso-occlusive pain episodes (VOE) cause severe pain in patients with sickle cell disease
(SCD). Vaso-occlusive events promote ischemia/reperfusion pathobiology that activates …

A MASP-like functions as PRR to regulate the mRNA expressions of inflammatory factors in the Pacific oyster Crassostrea gigas

P Wei, W Yang, W Wang, Y Li, X Yan, W Wu… - Fish & Shellfish …, 2023 - Elsevier
Mannose-binding lectin-associated serine protease (MASP) is a type of central serine
protease in the complement lectin pathway. In the present study, a MASP-like was identified …

Extracellular Vesicle Size Reveals Cargo Specific to Coagulation and Inflammation in Pediatric and Adult Sickle Cell Disease

K Thangaraju, S Setua, C Lisk… - Clinical and Applied …, 2023 - journals.sagepub.com
Aberrant coagulation in sickle cell disease (SCD) is linked to extracellular vesicle (EV)
exposure. However, there is no consensus on the contributions of small EVs (SEVs) and …

Acute sickle cell hepatopathy: A case report and literature review

M Hassanzadeh, ZM Sanat, S Khayatian… - Journal of the National …, 2024 - Elsevier
Sickle cell disease (SCD) is an inherited hemoglobinopathy with protean clinical
manifestations. The liver could be affected by various SCD-associated complications of an …