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filingDate 2021-05-25-04:00^^<http://www.w3.org/2001/XMLSchema#date>
grantDate 2022-02-08-04:00^^<http://www.w3.org/2001/XMLSchema#date>
publicationDate 2022-02-08-04:00^^<http://www.w3.org/2001/XMLSchema#date>
publicationNumber CN-113368247-B
titleOfInvention Application of HOIP inhibitor in preparation of medicine for treating type II human telangiectasia
abstract The invention discloses an application of a HOIP inhibitor in treating type II human telangiectasia (HHT 2). The binding domain and the binding mechanism of the ALK1 kinase and the HOIP are firstly disclosed, and the ALK1 mutant is found to be combined with the HOIP to enhance so as to obviously enhance the ubiquitination level. By inhibiting HOIP ubiquitin ligase activity, the ubiquitination level of the ALK1 mutant is reduced, so that an ALK1-Smad1/5 signal pathway is restored, and the symptom of HHT2 is relieved. The invention proves that the pathogenesis of HHT2 with ALK1 mutation is caused by reduction of ALK1 kinase enzyme activity, and the specific mutant of ALK1 can be inhibited through an HOIP inhibitor, so that the HHT2 can be treated, and the HOIP inhibitor can be a potential drug for treating HHT2 with specific ALK1 mutation.
priorityDate 2021-05-25-04:00^^<http://www.w3.org/2001/XMLSchema#date>
type http://data.epo.org/linked-data/def/patent/Publication

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