Results 11 to 20 of about 8,844 (195)

Conformational activation of ADAMTS13 [PDF]

Proceedings of the National Academy of Sciences of the United States of America, 2014
A disintegrin and metalloprotease with thrombospondin motifs 13 (ADAMTS13) is a metalloprotease that regulates von Willebrand factor (VWF) function. ADAMTS13-mediated proteolysis is determined by conformational changes in VWF, but also may depend on its ...
Kieron South, Brenda M Luken, James T B Crawley   +2 more
exaly   +3 more sources

Hereditary Thrombotic Thrombocytopenic Purpura Associated With Recurrent Strokes and Prominent Nervous System Involvement in a Young Chinese Female. [PDF]

Clin Case Rep
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, Wu J, Ming X, Zhang Q, Zhou D, Zheng R, Zhou M, Shang Z, Chen L, Zhu X, Xiao Y.   +10 more
europepmc   +2 more sources

Inherited ADAMTS13 mutations associated with Thrombotic Thrombocytopenic Purpura: a short review and update

Platelets, 2023
ADAMTS13 is a plasma metalloprotease with the primary function of cleaving VWF to maintain hemostasis. Circulating ADAMTS13 is in the closed conformation until blood vessel injury triggers a VWF-dependant activation to the open active form of the protein.
Zoe Markham-Lee, Neil V. Morgan, Jonas Emsley   +2 more
doaj   +1 more source

Characterization of ADAMTS13 and von Willebrand factor levels in septic and non-septic ICU patients.

PLoS ONE, 2021
Sepsis is a life-threatening disease characterized by excessive host response to infection that can lead to activation of the coagulation system. Von Willebrand Factor (VWF) and ADAMTS13 are important regulators of hemostasis and their dysregulation ...
Kanwal Singh, Andrew C Kwong, Hasam Madarati, Sharumathy Kunasekaran, Taylor Sparring, Alison E Fox-Robichaud, Patricia C Liaw, Colin A Kretz   +7 more
doaj   +1 more source

Localization of blood proteins thrombospondin1 and ADAMTS13 to cerebral corpora amylacea [PDF]

Neuropathology, 2009
Corpora amylacea (CA) have long been described in aging brains and in patients with neurodegenerative conditions, but their origins have been debated. It has been proposed that CA represent collections of nervous system breakdown products that accumulate within astrocytic cytoplasm.
Meng, He, Zhang, Xiaojie, Blaivas, Mila, Wang, Michael M.   +3 more
openaire   +3 more sources

Synonymous ADAMTS13 variants impact molecular characteristics and contribute to variability in active protein abundance

Blood Advances, 2022
The effects of synonymous single nucleotide variants (sSNVs) are often neglected since they do not alter protein primary structure. Nevertheless, there is growing evidence that synonymous variations may impact mRNA expression, protein conformation and activity which may lead to protein deficiency and disease manifestations.
Katarzyna Izabela Jankowska, Douglas Meyer, David Dillon Fisher Holcomb, Jacob Kames, Nobuko Hamasaki-Katagiri, Upendra K. Katneni, Ryan C. Hunt, Juan C. Ibla, Chava Kimchi-Sarfaty   +8 more
openaire   +2 more sources

Characterization of conformation-sensitive antibodies to ADAMTS13, the von Willebrand cleavage protease. [PDF]

PLoS ONE, 2009
BACKGROUND:The zinc metalloprotease ADAMTS13 is a multidomain protein that cleaves von Willebrand Factor (VWF) and is implicated in Thrombotic Thrombocytopenic Purpura (TTP) pathogenesis.
Zuben E Sauna, Chinyere Okunji, Ryan C Hunt, Tanvi Gupta, Courtni E Allen, Elizabeth Plum, Adam Blaisdell, Vahan Grigoryan, S Geetha, Robert Fathke, Kenji Soejima, Chava Kimchi-Sarfaty   +11 more
doaj   +1 more source

ADAMTS13 levels in a plasma-derived FVIII concentrate: A potential therapeutic option for patients with congenital thrombotic thrombocytopenic purpura

Thrombosis Update, 2022
Introduction: Treatment of congenital thrombotic thrombocytopenic purpura (cTTP), a disease characterized by the congenital deficiency of ADAMTS13, remains a challenge as there are no specific treatments available yet, other than therapy based on the use
Filippo Mori, Ilaria Nardini, Silvia Nannizzi, Roberto Crea, Prasad Mathew, Nicole Ziliotto, Alessandro Gringeri   +6 more
doaj   +1 more source

Characterization of coding synonymous and non-synonymous variants in ADAMTS13 using ex vivo and in silico approaches. [PDF]

PLoS ONE, 2012
Synonymous variations, which are defined as codon substitutions that do not change the encoded amino acid, were previously thought to have no effect on the properties of the synthesized protein(s).
Nathan C Edwards, Zachary A Hing, Avital Perry, Adam Blaisdell, David B Kopelman, Robert Fathke, William Plum, Jordan Newell, Courtni E Allen, Geetha S, Aaron Shapiro, Chinyere Okunji, Idit Kosti, Noam Shomron, Vahan Grigoryan, Teresa M Przytycka, Zuben E Sauna, Raheleh Salari, Yael Mandel-Gutfreund, Anton A Komar, Chava Kimchi-Sarfaty   +20 more
doaj   +1 more source

S109 Adamts13 protein levels are decreased in chronic thromboembolic pulmonary hypertension and implicated in its pathobiology [PDF]

Advances in understanding chronic thrombo-embolic disease and pulmonary hypertension, 2017
Introduction Chronic thromboembolic pulmonary hypertension (CTEPH) Results from failure of thrombus resolution following acute pulmonary embolism. Abnormalities in haemostasis are implicated in the pathobiology, including elevated levels of von Willebrand factor (VWF), which is normally regulated by ADAMTS13.
M Newnham, K South, M Bleda, J Cannon, S Gräf, C Hadinnapola, K Sheares, D Taboada, MR Wilkins, J Wharton, J Pepke-Zaba, M Laffan, DA Lane, M Toshner, NW Morrell   +14 more
openaire   +1 more source