Results 11 to 20 of about 5,761 (161)

Overview of ADAMTS13 Protein in Diagnosis and Patient Management of TTP [PDF]

Documenta Haematologica
The accurate diagnosis and effective management of Thrombotic Thrombocytopenic Purpura (TTP), a rare but life-threatening hematologic disorder, rely critically on the assessment of ADAMTS13 activity.
Georgiana GHERGHE, Valentina USCATESCU, Delia Codruta POPA, Diana PREDA, Didona VASILACHE, Mihai Emanuel HIMCINSCHI, Horia SANDU, Daniel CORIU   +7 more
doaj   +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

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

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 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

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

ADAMTS13 inhibits H2O2-induced human venous endothelial cell injury to attenuate deep-vein thrombosis by blocking the p38/ERK signaling pathway

Chinese Journal of Physiology, 2023
Deep vein thrombosis (DVT) is a common complication in hematologic malignancies and immunologic disorders. Endothelial cell injury and dysfunction comprise the critical contributor for the development of DVT.
Guangfeng Zheng, Qiang Zhang, Chuanyong Li, Weijian Fan, Zhichang Pan, Yuting Zhou, Yan Chen, Jianjie Rong   +7 more
doaj   +1 more source

Probing ADAMTS13 substrate specificity using phage display. [PDF]

PLoS ONE, 2015
Von Willebrand factor (VWF) is a large, multimeric protein that regulates hemostasis by tethering platelets to the subendothelial matrix at sites of vascular damage.
Karl C Desch, Colin Kretz, Andrew Yee, Robert Gildersleeve, Kristin Metzger, Nidhi Agrawal, Jane Cheng, David Ginsburg   +7 more
doaj   +1 more source