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ADAMTS13 information


ADAMTS13
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesADAMTS13, ADAM-TS13, ADAMTS-13, C9orf8, VWFCP, vWF-CP, ADAM metallopeptidase with thrombospondin type 1 motif 13
External IDsOMIM: 604134 MGI: 2685556 HomoloGene: 16372 GeneCards: ADAMTS13
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_139025
NM_139026
NM_139027
NM_139028

NM_001001322
NM_001290463
NM_001290464
NM_001290465

RefSeq (protein)

NP_620594
NP_620595
NP_620596

NP_001001322
NP_001277392
NP_001277393
NP_001277394

Location (UCSC)Chr 9: 133.41 – 133.46 MbChr 2: 26.86 – 26.9 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse

ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13)—also known as von Willebrand factor-cleaving protease (VWFCP)—is a zinc-containing metalloprotease enzyme that cleaves von Willebrand factor (vWf), a large protein involved in blood clotting. It is secreted into the blood and degrades large vWf multimers, decreasing their activity, hence ADAMTS13 acts to reduce thrombus formation.[5]

  1. ^ a b c ENSG00000281244 GRCh38: Ensembl release 89: ENSG00000160323, ENSG00000281244 – Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000014852 – Ensembl, May 2017
  3. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. ^ Levy GG, Motto DG, Ginsburg D (July 2005). "ADAMTS13 turns 3". Blood. 106 (1): 11–7. doi:10.1182/blood-2004-10-4097. PMID 15774620. S2CID 25645477.

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ADAMTS13

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ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13)—also known as von Willebrand factor-cleaving protease (VWFCP)—is...

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Thrombotic thrombocytopenic purpura

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underlying mechanism typically involves antibodies inhibiting the enzyme ADAMTS13. This results in decreased break down of large multimers of von Willebrand...

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Von Willebrand factor

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must partially unfold to expose the buried cleavage site for the specific ADAMTS13 protease that inactivates VWF by making much smaller multimers. The partial...

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ADAMTS13 endopeptidase

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ADAMTS13 endopeptidase (EC 3.4.24.87, ADAMTS VWF cleaving metalloprotease, ADAMTS-13, ADAMTS13, vWF-cleaving protease, VWF-CP, vWF-degrading protease,...

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Apadamtase alfa

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public domain. "Takeda's Adzynma (ADAMTS13, recombinant-krhn) Approved by U.S. FDA as the First and Only Recombinant ADAMTS13 Enzyme Replacement Therapy for...

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Stroke

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factor levels increase the risk of first ischemic stroke: influence of ADAMTS13, inflammation, and genetic variability". Stroke. 37 (11): 2672–7. doi:10...

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ABO blood group system

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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...

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ADAMTS

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thrombocytopenic purpura arise from autoantibody-mediated inhibition of ADAMTS13. Like ADAMs, the name of the ADAMTS family refers to its disintegrin and...

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Inherited thrombotic thrombocytopenic purpura

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activity of the von Willebrand factor-cleaving protease ADAMTS13. Hereditary TTP, caused by ADAMTS13 gene mutations, is much less common. Congenital or inherited...

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Thrombotic microangiopathy

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Congenital and idiopathic TTP are generally associated with deficiencies in ADAMTS13, a zinc metalloprotease responsible for cleaving Very Large vWF Multimers...

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Oxymorphone

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therapeutic plasma exchange, as for TTP. Unlike TTP, no deficient ADAMTS13 activity nor anti-ADAMTS13 antibody was found indicating a thrombotic microangiopathy...

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Atypical hemolytic uremic syndrome

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characterized by mutations in the ADAMTS13 gene leading to severe ADAMTS13 deficiency. This congenital cause of ADAMTS13 deficiency is called Upshaw-Schülman...

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Chromosome 9

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group glycosyltransferases ACTL7A: encoding protein Actin-like protein 7A ADAMTS13: ADAM metallopeptidase with thrombospondin type 1 motif, 13 AIF1L: allograft...

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List of autoimmune diseases

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(adults), 50 per 100,000 (children) Thrombotic thrombocytopenic purpura ADAMTS13 autoantibodies Confirmed 1-2 per million Antiphospholipid syndrome Antiphospholipid...

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Thrombospondin

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the treatment of several types of cancer. ADAMTS1; ADAMTS10; ADAMTS12; ADAMTS13; ADAMTS14; ADAMTS15; ADAMTS16; ADAMTS17; ADAMTS18; ADAMTS19; ADAMTS2; ADAMTS20;...

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Von Willebrand disease

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electrophoresis due to impaired multimer assembly or increased susceptibility to ADAMTS13 (a protease that cleaves VWF). Factor VIII activity can be normal or low...

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Epitope mapping

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nearly single amino acid residue resolution reveals novel exosites on ADAMTS13 critical for substrate recognition and mechanism of autoimmune thrombotic...

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Eli Moschcowitz

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doctors who performed the full autopsy. Thrombotic thrombocytopenic purpura ADAMTS13 John Vivian Dacie Marcus, Jacob Rader & Daniels, Judith M. (Ed.): The concise...

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Hemolytic jaundice

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which the reduced activity of the von Willebrand factor-cleaving protease ADAMTS13 causes a thrombotic microangiopathy. This disease, acquired or hereditary...

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ADAMTS2

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and skin), which causes the signs and symptoms of the disorder. ADAMTS5 ADAMTS13 GRCm38: Ensembl release 89: ENSMUSG00000036545 – Ensembl, May 2017 "Human...

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Proteases in angiogenesis

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divided into three groups: procollagen aminopeptidase, aggrecanase, and ADAMTS13 which cleaves von Willebrand factor. Unlike with MMPs, TIMPs are more selective...

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Disintegrin

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families, which include important protease enzymes. The secreted protease ADAMTS13, found in serum, cleaves Von Willebrand factor and acts as a natural, endogenous...

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List of OMIM disorder codes

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hyperhomocysteinemic; 236200; CBS Thrombotic thrombocytopenic purpura, familial; 274150; ADAMTS13 Thryoid dyshormonogenesis 6; 607200; DUOX2 Thyroid carcinoma, follicular;...

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