Tsis ntev los no, cov kws tshawb fawb los ntawm Utrecht University Medical Center tau luam tawm cov ntaub ntawv tshawb fawb hauv Nature Nature Cardiovascular Research txoj cai: Kev kho mob ntawm AAV-mediated restoration ntawm PKP2 hauv arrhythmogenic cardiomyopathy.
Txoj kev tshawb no, siv adeno-associated virus (AAV)-xa PKP2 noob kev kho mob, nteg lub hauv paus rau kev kho noob rau arrhythmogenic cardiomyopathy (ACM), kab mob plawv, uas yuav raug sim hauv ntau qhov kev sim tshuaj hauv Teb Chaws Asmeskas xyoo 2024.
Arrhythmogenic cardiomyopathies (ACM) feem ntau yog vim muaj kev hloov pauv hauv cov noob muaj feem xyuam rau cov desmosome, uas yog lub luag haujlwm rau kev sib txuas cov neeg nyob sib ze cardiomyocytes; Tsis tsuas yog lawv muab cov qauv kev sib txuas, tab sis lawv kuj xyuas kom meej tias cardiomyocytes cog lus nyob rau hauv synchrony, tso cai rau lub plawv twj tso kua mis hauv kev sib koom tes.
Feem ntau hom ntawm arrhythmogenic cardiomyopathy (ACM) yog kev hloov pauv hauv PKP2 noob, uas encodes cov protein plakophilin-2, ib qho tseem ceeb ntawm cov nplej nplej. Cov neeg mob uas muaj kev hloov pauv hauv cov noob no feem ntau muaj qis qis ntawm plakophilin-2 protein nyob rau hauv lawv cov cardiomyocytes, uas ua rau tsis muaj zog kev sib txuas ntawm cardiomyocytes, ua rau nws nyuaj rau lawv ua hauj lwm hauv synchrony thiab ua rau kev loj hlob ntawm arrhythmias.
Yog li, pab pawg tshawb fawb tau txiav txim siab tsim cov tshuaj kho noob hom phiaj kev hloov pauv hauv PKP2 noob los kho arrhythmogenic cardiomyopathy (ACM) ntawm nws cov hauv paus. Qhia qhov tseeb PKP2 noob rau hauv cov kab mob cardiomyocytes muaj peev xwm rov qab tau plakophilin-2 cov protein kom zoo li qub, yog li txhim kho bridging granules thiab txo cov arrhythmias hauv cov neeg mob no.
Siv ntau lub chaw kuaj mob ntawm arrhythmogenic cardiomyopathy (ACM), pab pawg tau pom tias kev xa cov noob PKP2 raug rau tib neeg cardiomyocytes tau los ntawm cov qia hlwb rov qab plakophilin-2 qib thiab kuj txhim kho lawv cov sodium transduction, uas yog qhov tseem ceeb rau kev cog lus ntawm cardiomyocytes.
Tom qab ntawd, pab pawg tau lees paub qhov kev kho mob tau zoo dua qub ntawm myocardial contractility nyob rau hauv chav kuaj-cultured engineered human cardiomyocytes. Thaum kawg, pab pawg tau lees paub ntxiv txog qhov cuam tshuam ntawm PKP2 noob kev kho mob hauv tus qauv nas ntawm arrhythmogenic cardiomyopathy (ACM), uas tau ua tiav kev txhim kho kev rov qab los ntawm kev sib txuas thiab kev ua haujlwm ntawm lub plawv hauv tus qauv nas.
Tom qab txhawb kev nce qib hauv kev tshawb fawb hauv tsev kho mob, cov kauj ruam tom ntej yog los tshawb xyuas cov kev kho mob muaj peev xwm ntawm qhov kev kho cov noob no hauv cov neeg mob arrhythmogenic cardiomyopathy (ACM) uas muaj kev hloov pauv hauv PKP2 noob.
Eirini Kyriakopoulou, thawj tus kws sau ntawv, tau hais tias peb lub tuam txhab hauv Tebchaws Meskas tau tshaj tawm tias lawv yuav pib kev sim tshuaj rau xyoo tom ntej txhawm rau ntsuas qhov ua tau zoo ntawm kev kho cov noob no hauv cov neeg mob. Thaum arrhythmogenic cardiomyopathy (ACM) tau nce mus rau qhov chaw uas ib feem ntawm lub plawv cov leeg tau hloov los ntawm cov ntaub so ntswg rog, nws tsis paub tseeb tias txoj hauv kev tuaj yeem thim rov qab kev puas tsuaj rau lub plawv cov leeg uas twb muaj lawm. Txawm li cas los xij, nws yuav txaus los tiv thaiv tus kab mob thaum ntxov mus rau theem hnyav dua.
Txawm hais tias cov txiaj ntsig ntawm kev sim tshuaj ua ntej thiab kev sim tshuaj tom ntej tau cog lus zoo rau arrhythmogenic cardiomyopathy (ACM), qhov tseeb kev lag luam ntawm kev kho yuav yog ntau xyoo. Ntxiv nrog rau kev lees paub nws qhov kev ua tau zoo hauv tib neeg cov neeg mob, txhua qhov kev txhawj xeeb txog kev nyab xeeb yuav tsum tau hais thiab tshem tawm ua ntej thov kev kho mob.
