Zatsopano zochokera ku Italy motsutsana ndi matenda a Hurler
Zatsopano zofunika zachipatala zothana ndi matenda a Hurler
Kodi Hurler Syndrome ndi chiyani?
Chimodzi mwa matenda osowa kwambiri amene angayambe ana ndi Matenda a Hurler, mwaukadaulo wotchedwa “mukopolisaccharidosis type 1H“. Izi osowa matenda amakhudza Mwana mmodzi pa 1 aliwonse kubadwa mwatsopano. Zimaphatikizapo kusowa kwa puloteni inayake yomwe imayambitsa kusokoneza shuga, glycosaminoglycans. Kuchulukana kwa mashugawa kumayambitsa kuwonongeka kwa ma cell, kusokoneza kukula komanso kukulitsa chidziwitso cha ana.
Mwatsoka, zotsatira zake ndi zoipa, ndipo imfa imatha kuchitika munthu akamakula, makamaka chifukwa cha matenda a mtima kapena kupuma.
Malo atsopano azachipatala
Kale mu 2021, kafukufuku wa San Raffaele Telethon Institute for Gene Therapy anali atawonetsa zotsatira zabwino. Mchitidwewu umaphatikizapo kupereka ndondomeko yolondola ya chidziwitso cha majini chofunikira kuti apange enzyme yomwe ikusowa.
Chapadera cha chithandizo chagona pakugwiritsa ntchito, posintha ma cell a hematopoietic stem cell, s.ome ma vector otengedwa ku HIV, kachilombo koyambitsa Edzi. Kuyenera kudziwidwa kuti mochulukirachulukira, magawo ang'onoang'ono a mndandanda wapachiyambi akugwiritsidwa ntchito m'munda wa chithandizo cha majini ku matenda osowa.
Kafukufuku watsopano wofalitsidwa mu nyuzipepala ya JCI Insight, yochitidwa ndi ofufuza apadziko lonse lapansi motsogozedwa ndi Sapienza University of Rome ndi Tettamanti Foundation ya Monza, ndi zopereka zochokera ku Irccs San Gerardo dei Tintori Foundation ya Monza ndi University of Milano-Bicocca, yalola kuti labotale ipangidwe. organoid ya fupa, mtundu wosavuta komanso wa mbali zitatu wa minofu yomwe imapanga mafupa ndi chichereŵechereŵe m'thupi la munthu.
Izi zitha kuwala kwatsopano pa Hurler syndrome.
Adafunsidwa ndi Ansa, Madokotala Serafini ndi Riminucci, olemba anzawo a kafukufukuyu ndi Samantha Donsante wa ku Sapienza ndi Alice Pievani wa Tettamanti Foundation monga otsogolera osayina, adanena kuti kupangidwa kwa organoid sikungotsegula. zitseko zatsopano zothana ndi matenda a Hurler komanso kuzama kafukufuku wa chithandizo cha matenda ena aakulu chibadwa.
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