Results 71 to 80 of about 5,721 (151)
ABSTRACT Osteosarcoma (OS) is an aggressive primary bone tumour with high metastatic potential. Current treatments including surgery and chemotherapy are limited by side effects and chemoresistance, underscoring the need for novel therapies. This study aimed to investigate the antitumor potential of resveratrol (RSV), a natural polyphenol, as a novel ...
Raffaella De Pace +6 more
wiley +1 more source
Nonsense-mediated mRNA decay in the ADAMTS13 gene caused by a 29-nucleotide deletion
Background In mammalian cells a regulatory mechanism, known as nonsense-mediated mRNA decay, degrades mRNA harboring premature termination codons. This mechanism is intron-dependent and functions as a quality control mechanism to eliminate abnormal ...
Isabella Garagiola +5 more
doaj +1 more source
Unraveling antibody-induced structural dynamics in the ADAMTS13 CUB1-2 domains via HDX-MS
: Allosteric regulation of ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type-1 motif, member 13) activity involves an interaction between its spacer (S) and 2 complement C1r/C1s, Uegf and BMP1 (CUB; CUB1-2) domains to keep the enzyme
Quintijn Bonnez +8 more
doaj +1 more source
Successful Treatment of Refractory Immune Thrombotic Thrombocytopenic Purpura with Daratumumab
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare life-threatening disease manifested by thrombocytopenia, microangiopathic hemolytic anemia, and impaired functions of organs and systems due to the thrombosis of small arteries.
E. E. Klebanova +5 more
doaj +1 more source
Moschcowitz Disease in pediatric. [PDF]
Hemichorea Moskowitz disease is a rare hematological disorder known to be an autoimmune disease in most patients. Most cases of thrombotic thrombopenic purpura (TTP) are caused by problems with an enzyme or protein in the blood called ADAMTS13.
Samira AGGOUNE, Mohamed Faouzi SEDDIKI
doaj +1 more source
Thrombotic Thrombocytopenic Purpura (TTP) is a rare disease characterized by a severe deficiency of ADAMTS13, the specific protease that cleaves von Willebrand factor. The congenital form of TTP (cTTP) results from pathogenic variants of the ADAMTS13 gene.
Joly, Bérangère S. +8 more
openaire +2 more sources
Thrombotic Thrombocytopenic Purpura in Oman: Disease Burden and Outcomes
Objectives: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening autoimmune disorder; limited information about this disease is available from the Middle East.
Samata Al Dowaiki +4 more
doaj +1 more source
Introduction Since the first description of Thrombotic Thrombocytopenic Purpura, caused by a severe deficiency of ADAMTS13, plasma exchange and immunosuppression have become standard treatments, allowing to decrease its high mortality rate.
Daniel Cathalifaud +6 more
doaj +1 more source
Background: Multimarker panels of blood-borne biomarkers could aid in shortening the diagnostic delay of pulmonary arterial hypertension (PAH). Research Question: Can any of the 61 proteins included in the study, related to pathways known to be involved ...
Kriss Kania, MD +6 more
doaj +1 more source
PROTEOLYTIC PROCESSING OF VON WILLEBRAND FACTOR BY ADAMTS13 AND LEUKOCYTE PROTEASES-rev1-MJHID-2013
ADAMTS13 is a 190 kDa zinc protease encoded by a gene located on chromosome 9q34. This protease specifically hydrolyzes von Willebrand factor (VWF) multimers, thus causing VWF size reduction.
Raimondo De Cristofaro
doaj

