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Metalloprotease enzyme
ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13)—also known as von Willebrand factor-cleaving protease (VWFCP)—is
ADAMTS13
Medical condition
underlying mechanism typically involves antibodies inhibiting the enzyme ADAMTS13. This results in decreased break down of large multimers of von Willebrand
Thrombotic thrombocytopenic purpura
Thrombotic_thrombocytopenic_purpura
Medical condition
complex blood coagulation disease. USS is caused by the absence of the ADAMTS13 protease resulting in the persistence of ultra large von Willebrand factor
Upshaw–Schulman_syndrome
Medication
Manufacturing Austria AG. The active substance of Adzynma is recombinant ADAMTS13 (rADAMTS13). Apadamtase alfa was approved for medical use in the European Union
Apadamtase_alfa
Medical condition
first-line treatment. This treatment replenishes the body with ADAMTS13 while filtering out anti-ADAMTS13 antibodies. Glucocorticoids are also commonly administered
Thrombocytopenic_purpura
Class of enzymes
ADAMTS13 endopeptidase (EC 3.4.24.87, ADAMTS VWF cleaving metalloprotease, ADAMTS-13, ADAMTS13, vWF-cleaving protease, VWF-CP, vWF-degrading protease,
ADAMTS13_endopeptidase
Mammalian protein involved in blood clotting
osteoprotegerin. The A2 domain: Unfolds to expose the cleavage site for ADAMTS13 protease, which cleaves VWF into smaller multimers. Unfolding is influenced
Von_Willebrand_factor
Death of a region of brain cells due to poor blood flow
factor levels increase the risk of first ischemic stroke: influence of ADAMTS13, inflammation, and genetic variability". Stroke. 37 (11): 2672–7. doi:10
Stroke
Classification of blood types
Cys1584 variant of vWF (an amino acid polymorphism in VWF): the gene for ADAMTS13 (vWF-cleaving protease) maps to human chromosome 9 band q34.2, the same
ABO_blood_group_system
Medical condition
into its active state, it is degraded by its natural catabolic enzyme ADAMTS13, rendering it incapable of binding the collagen at an injury site. As the
Heyde's_syndrome
Family of protease enzymes
thrombocytopenic purpura arise from autoantibody-mediated inhibition of ADAMTS13. Like ADAMs, the name of the ADAMTS family refers to its disintegrin and
ADAMTS
Disease of blood and kidneys after bacterial infection
Shiga-toxin/EHEC test confirms a cause for STEC-HUS, and severe ADAMTS13 deficiency (i.e., ≤5% of normal ADAMTS13 levels) confirms a diagnosis of TTP. The effect of
Hemolytic–uremic_syndrome
Genetic disorder
activity of the von Willebrand factor-cleaving protease ADAMTS13. Hereditary TTP, caused by ADAMTS13 gene mutations, is much less common. Congenital or inherited
Inherited thrombotic thrombocytopenic purpura
Inherited_thrombotic_thrombocytopenic_purpura
Opioid analgesic drug
therapeutic plasma exchange, as for TTP. Unlike TTP, no deficient ADAMTS13 activity nor anti-ADAMTS13 antibody was found indicating a thrombotic microangiopathy
Oxymorphone
Life-threatening immune-related blood disease
characterized by mutations in the ADAMTS13 gene leading to severe ADAMTS13 deficiency. This congenital cause of ADAMTS13 deficiency is called Upshaw-Schulman
Atypical hemolytic uremic syndrome
Atypical_hemolytic_uremic_syndrome
Medical condition
Congenital and idiopathic TTP are generally associated with deficiencies in ADAMTS13, a zinc metalloprotease responsible for cleaving Very Large vWF Multimers
Thrombotic_microangiopathy
85: S2P endopeptidase EC 3.4.24.86: ADAM 17 endopeptidase EC 3.4.24.87: ADAMTS13 endopeptidase EC 3.4.25.1: proteasome endopeptidase complex EC 3.4.25.2:
List_of_EC_numbers_(EC_3)
Protein domain
the treatment of several types of cancer. ADAMTS1; ADAMTS10; ADAMTS12; ADAMTS13; ADAMTS14; ADAMTS15; ADAMTS16; ADAMTS17; ADAMTS18; ADAMTS19; ADAMTS2; ADAMTS20;
Thrombospondin
American doctor (1879–1964)
doctors who performed the full autopsy. Thrombotic thrombocytopenic purpura ADAMTS13 John Vivian Dacie Marcus, Jacob Rader & Daniels, Judith M. (Ed.): The concise
Eli_Moschcowitz
Medical condition
secreted by endothelial cells, the multimers are cleaved by the enzyme ADAMTS13 and vWF circulates in the plasma in a coiled and inactive form. When there
Von_Willebrand_disease
Protein-coding gene in humans
and skin), which causes the signs and symptoms of the disorder. ADAMTS5 ADAMTS13 GRCm38: Ensembl release 89: ENSMUSG00000036545 – Ensembl, May 2017 "Human
ADAMTS2
Topics referred to by the same term
factor-cleaving protease, a.k.a. ADAMTS13, a zinc-containing metalloprotease enzyme Von Willebrand factor cleaving protease, a.k.a. ADAMTS13 endopeptidase, an enzyme
Willebrand_(disambiguation)
Identifying the binding site of an antibody on its target antigen
nearly single amino acid residue resolution reveals novel exosites on ADAMTS13 critical for substrate recognition and mechanism of autoimmune thrombotic
Epitope_mapping
(adults), 50 per 100,000 (children) Thrombotic thrombocytopenic purpura ADAMTS13 autoantibodies Confirmed 1-2 per million Antiphospholipid syndrome Antiphospholipid
List_of_autoimmune_diseases
divided into three groups: procollagen aminopeptidase, aggrecanase, and ADAMTS13 which cleaves von Willebrand factor. Unlike with MMPs, TIMPs are more selective
Proteases_in_angiogenesis
Human chromosome
group glycosyltransferases ACTL7A: encoding protein Actin-like protein 7A ADAMTS13: ADAM metallopeptidase with thrombospondin type 1 motif, 13 AIF1L: allograft
Chromosome_9
Q9P2N4 280 ADAMTS10 HGNC:13201; Q9H324 281 ADAMTS12 HGNC:14605; P58397 282 ADAMTS13 HGNC:1366; Q76LX8 283 ADAMTS14 HGNC:14899; Q8WXS8 284 ADAMTS15 HGNC:16305;
List of human protein-coding genes 1
List_of_human_protein-coding_genes_1
Type of jaundice
which the reduced activity of the von Willebrand factor-cleaving protease ADAMTS13 causes a thrombotic microangiopathy. This disease, acquired or hereditary
Hemolytic_jaundice
Instance of defined set in Homo sapiens with Reactome ID (R-HSA-6806560)
syndrome-associated Shiga toxins promote endothelial-cell secretion and impair ADAMTS13 cleavage of unusually large von Willebrand factor multimers". Blood. 106
AP-1_transcription_factor
Medical condition
anti-clotting factor of the body (specific von Willebrand factor-cleaving protease ADAMTS13). Rarely, certain antibodies cause agglutination (clumping), in cold temperatures
Occlusive_vasculopathy
Proteins from viper venom inhibiting platelets aggregation
families, which include important protease enzymes. The secreted protease ADAMTS13, found in serum, cleaves Von Willebrand factor and acts as a natural, endogenous
Disintegrin
hyperhomocysteinemic; 236200; CBS Thrombotic thrombocytopenic purpura, familial; 274150; ADAMTS13 Thyroid dyshormonogenesis 6; 607200; DUOX2 Thyroid carcinoma, follicular;
List_of_OMIM_disorder_codes
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ADAMTS13
ADAMTS13
ADAMTS13
ADAMTS13
ADAMTS13
ADAMTS13
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