The biology of SCUBE

YC Lin, BK Sahoo, SS Gau, RB Yang - Journal of biomedical science, 2023 - Springer
Abstract The SCUBE [Signal peptide-Complement C1r/C1s, Uegf, Bmp1 (CUB)-Epithelial
growth factor domain-containing protein] family consists of three proteins in vertebrates …

TTP: From empiricism for an enigmatic disease to targeted molecular therapies

NAG Graça, BS Joly, J Voorberg… - British journal of …, 2022 - Wiley Online Library
The 100th anniversary of the first description of Thrombotic Thrombocytopenic Purpura
(TTP) as a disease by Dr. Eli Moschcowitz approaches. For many decades, TTP remained …

ADAMTS13 and non-ADAMTS13 biomarkers in immune-mediated thrombotic thrombocytopenic purpura

Q Bonnez, K Sakai, K Vanhoorelbeke - Journal of Clinical Medicine, 2023 - mdpi.com
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare medical emergency
for which a correct and early diagnosis is essential. As a severe deficiency in A Disintegrin …

Thrombotic microangiopathies: an illustrated review

MY Abou‐Ismail, S Kapoor… - … and Practice in …, 2022 - Wiley Online Library
The thrombotic microangiopathies (TMAs) are a heterogenous group of disorders with
distinct pathophysiologies that cause occlusive microvascular or macrovascular thrombosis …

Open ADAMTS-13 conformation index predicts earlier relapse in immune-mediated thrombotic thrombocytopenic purpura

L De Waele, K Sakai, I Mancini, G Sinkovits… - Journal of Thrombosis …, 2024 - Elsevier
Background ADAMTS-13 adopts an open conformation in patients with immune-mediated
thrombotic thrombocytopenic purpura (iTTP) in acute phase while being closed in healthy …

ADAMTS13 conformation and immunoprofiles in Japanese patients with immune-mediated thrombotic thrombocytopenic purpura

K Sakai, M Matsumoto, L De Waele… - Blood …, 2023 - ashpublications.org
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is an ultrarare thrombotic
disease caused by autoantibody-induced ADAMTS13 deficiency. Open ADAMST13 …

ADAMTS13 antibody and inhibitor assays

GW Moore, H Vetr, NB Binder - Hemostasis and Thrombosis: Methods and …, 2023 - Springer
A finding of an ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type
1 motif, member 13) activity level of< 10% of normal is usually sufficient to distinguish …

Frontiers in pathophysiology and management of thrombotic thrombocytopenic purpura

M Kubo, M Matsumoto - International Journal of Hematology, 2023 - Springer
Thrombotic thrombocytopenic purpura (TTP) is a fatal disease in which platelet-rich
microthrombi cause end-organ ischemia and damage. TTP is caused by markedly reduced …

Conformational plasticity of ADAMTS13 in hemostasis and autoimmunity

B Ercig, T Arfman, J Hrdinova, K Wichapong… - Journal of Biological …, 2021 - ASBMB
A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13
(ADAMTS13) is a multidomain metalloprotease for which until now only a single substrate …

Anti-ADAMTS13 autoantibodies: from pathophysiology to prognostic impact—A review for clinicians

C Dainese, F Valeri, B Bruno, A Borchiellini - Journal of Clinical Medicine, 2023 - mdpi.com
Thrombotic thrombocytopenic purpura (TTP) is a fatal disease in which platelet-rich
microthrombi cause end-organ ischemia and damage. TTP is caused by markedly reduced …