Adamts13

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

ADAMTS 13; ADAMTS-13; A disintegrin and metalloprotease with thrombospondin-1-like domains

Definition
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ADAMTS 13 is a zinc-containing metalloprotease that cleaves the von Willebrand factor (vWF). ADAMTS13 splits the vWF multimer into smaller sections. ADAMTS 13 thus prevents the accumulation of large multimers (UL-vWF). A reduced concentration of ADAMTS13 (<5-10%) leads to high UL-vWF levels in the blood plasma and thus to spontaneous platelet activation with thrombus formation. These thrombi consist of large Von Willebrand factor aggregates and activated platelets. Vascular occlusion leads to ischemia of the downstream tissues.

A strongly reduced activity (< 5-10 %) of metalloprotease is specific for the thrombocytopenic purpura (M31.1).

In the secondary forms ADAMTS13 is inconspicuous or slightly decreased.

In the relatively frequent autoimmunological (antibody formation), acquired form, ADAMTS13 antibodies are detectable.

General information
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ADAMTS-13 splits the vWF multimer into smaller sections and thus prevents the formation of larger aggregates (UL-vWF). A reduced activity of ADAMTS13 (<5-10%) leads to high UL-vWF levels in the blood plasma and thus to spontaneous platelet activation. These thrombi consist of large Von Willebrand factor complexes and activated platelets. The thrombotic vascular occlusion leads to ischemia of the downstream tissues (in the skin to necrotizing purpura).

A strongly reduced activity (< 5-10 %) of the protease is specific for a thrombocytic-thrombocytopenic purpura (M31 .1). In the secondary forms, ADAMTS13 is inconspicuous or slightly decreased. In the relatively frequent autoimmunological (antibody formation) acquired form, ADAMTS13 antibodies are detectable.

Note(s)
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Furthermore, a homologous protein subfamily was identified, the ADAMTS-like proteins, which do not have enzymatic functions. Of these, the ADAMTS-like protein 5 was identified as an autoantigen of melanocytes. It seems to play a role in the pathogenesis of psoriasis.

Literature
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  1. Mariotte E et al (2016) French Reference Center for Thrombotic Microangiopathies. Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy. Lancet Haematol 3:e237-245.
  2. Prince JC (2016) Autoimmune reactions in psoriasis. Dermatologist 67: 432-437
  3. Tersteeg C et al (2016) ADAMTS13 and anti-ADAMTS13 autoantibodies in thrombocytopenic purpura - current perspectives and new treatment strategies. Expert Rev Hematol 9: 209-221.
  4. Thomas MR et al (2015) Pathogenicity of anti-ADAMTS13 autoantibodies in acquired thrombocytopenic purpura. EBioMedicine. 2: 940-950.

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Last updated on: 29.10.2020