MITOXONDRIAL KASALLIKLAR VA GIPOENERGIK HOLATLAR
Keywords:
Mitoxondriya, oksidlovchi fosforlanish, ATP, gipoenergik holat, mtDNK, metabolik kasalliklar.Abstract
Mitoxondrial kasalliklar – hujayra energetikasining asosiy markazi bo‘lgan mitoxondriyadagi DNK mutatsiyalari yoki nuklear DNKdagi o‘zgarishlar tufayli yuzaga keladigan irsiy-metabolik buzilishlar guruhidir. Ushbu patologiyalar ATP sintezining keskin kamayishi, oksidlovchi fosforlanish jarayonining izdan chiqishi va to‘qimalarning energiyaga bo‘lgan ehtiyoji ta’minlanmasligi natijasida rivojlanadi. Kam energiyali (gipoenergik) holatlar mitoxondrial kasalliklarning markaziy klinik xususiyati bo‘lib, mushak zaifligi, nevrologik simptomlar, kardial yetishmovchilik, endokrin buzilishlar va ko‘p organli shikastlanish bilan kechadi. Ushbu maqolada mitoxondrial kasalliklarning etiologiyasi, patogenezi, asosiy klinik ko‘rinishlari hamda diagnostika va davolash yondashuvlari yoritildi.
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