ADAMTS13 Deficiency Worsens Colitis and Exogenous ADAMTS13 Administration Decreases Colitis Severity in Mice [PDF]
Background Inflammatory bowel disease (IBD) affects 1.6 million people in the United States. IBD is associated with an increased risk of thrombosis, which rises with disease activity.
Naamah L. Zitomersky +7 more
doaj +3 more sources
Hereditary Thrombotic Thrombocytopenic Purpura Associated With Recurrent Strokes and Prominent Nervous System Involvement in a Young Chinese Female. [PDF]
ABSTRACT Hereditary thrombotic thrombocytopenic purpura (TTP) is a rare autosomal recessive inherited disease caused by an ADAMTS1 gene mutation, resulting in absence or severe deficiency of plasma ADAMTS13 activity. The common causes include infection, inflammation, or pregnancy.
Liu W +10 more
europepmc +2 more sources
The VWF/ADAMTS13 axis in the antiphospholipid syndrome: ADAMTS13 antibodies and ADAMTS13 dysfunction [PDF]
SummaryAutoantibodies to ADAMTS13 (a disintegrin‐like and metalloprotease with thrombospondin type I motif, member 13) play an important role in the development of microthrombosis in thrombotic thrombocytopenic purpura (TTP). In severe cases of antiphospholipid syndrome (APS), microthrombosis can occur similar to that seen in TTP, suggesting possible ...
S K, Austin +5 more
openaire +2 more sources
ADAMTS13 activity and inhibitor [PDF]
AbstractThrombotic thrombocytopenic purpura (TTP) is often associated with acquired or congenital deficiency of the von Willebrand factor‐cleaving metalloprotease, ADMATS13 (Lammle B et al., J Thromb Haemost 2005;3:1663‐1675; Schneppenheim et al., Blood 2003;101:1845‐1850).
Adriana, Doldan-Silvero +4 more
openaire +2 more sources
Mechanisms of ADAMTS13 regulation
Recombinant ADAMTS13 is currently undergoing clinical trials as a treatment for hereditary thrombotic thrombocytopenic purpura, a lethal microvascular condition resulting from ADAMTS13 deficiency. Preclinical studies have also demonstrated its efficacy in treating arterial thrombosis and inflammation without causing bleeding, suggesting that ...
Veronica DeYoung +2 more
openaire +2 more sources
Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors : a single-institution experience [PDF]
Thrombotic microangiopathies are rare conditions characterized by microangiopathic hemolytic anemia, microthrombi, and multiorgan insult. The disorders, which include hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, are often acute and ...
Catlow, Jamie +4 more
core +1 more source
A diagnosis of thrombotic thrombocytopenic purpura (TTP) is confirmed by a severe deficiency (
Chiara Vendramin +3 more
doaj +1 more source
Diagnosis of thrombotic thrombocytopenic purpura
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease, disease, characterised by microangiopathic hemolytic anaemia, consumption thrombocytopenia, and organ dysfunction.
G. M. Galstyan, E. E. Klebanova
doaj +1 more source
The ADAMTS (A Disintegrin and Metalloproteinase with Thrombospondin motifs) family [PDF]
The ADAMTS (A Disintegrin and Metalloproteinase with Thrombospondin motifs) enzymes are secreted, multi-domain matrix-associated zinc metalloendopeptidases that have diverse roles in tissue morphogenesis and patho-physiological remodeling, in ...
A Colige +145 more
core +1 more source
The class I scavenger receptor CD163 promotes internalization of ADAMTS13 by macrophages
: Internalization of ADAMTS13 by macrophages may contribute to its clearance from the circulation. Here we investigated endocytic mechanisms that contribute to the uptake of ADAMTS13 by macrophages. Human monocyte-derived macrophages were used to monitor
Fabian C. Verbij +12 more
doaj +1 more source

