Alaabta

Ururinta nukliyeerka ee qaabka mutant ee borotiinka nukliyeerka ee Lamin-A, oo loo yaqaan Progerin (PG) ama Lamin AΔ50, waxay ku dhacdaa Hutchinson-Gilford Progeria Syndrome (HGPS) ama Progeria, cudur gabow ah oo la dedejiyey.Mid ka mid ah calaamadaha ugu muhiimsan ee xanuunkan hidde-sideyaasha ah waa luminta dufanka hoose ee maqaarka sababtoo ah lipodystrophy cajiib ah. Waxaan si adag u kicinnay muujinta PG iyo GFP-Green Fluorescent Protein- sida xakamaynta unugyada 3T3L1 iyadoo la adeegsanayo habka lentiviral si loo barto Saamaynta muujinta PG ee awoodda kala-soocidda ee xariiqda unugyada, mid ka mid ah moodooyinka adipogenic aadka loo isticmaalo.Proteomics tirada (iTRAQ) ayaa loo sameeyay si loo darso saamaynta ururinta PG.Dhawr ka mid ah borotiinada la habeeyey ayaa lagu ansaxiyay difaac-ilaaliye iyo PCR-waqtiga-dhabta ah.Shaqada Mitochondrial waxaa lagu falanqeeyay iyadoo la cabbirayo a) dhaqdhaqaaqa asaasiga ah ee mitochondrial, b) wax soo saarka superoxide anion iyo c) waxtarka shakhsi ahaaneed ee kakanaanta kala duwan ee silsiladda neefsiga. Waxaan ogaanay in muujinta xad-dhaafka ah ee PG ee bixinta hidda-wadaha lentivi ay keento a hoos u dhaca heerka fidinta iyo cilladaha awoodda adipogenic marka la barbardhigo xakamaynta.Falanqaynta borotiinka tirooyinka ayaa muujisay 181 borotiinno si weyn (p<0.05) lagu beddelay PG-muujinta preadipocytes.Hawlaha mitochondrial ayaa daciifay unugyada PG-muujinta.Gaar ahaan, waxaan ogaanay kororka dhaqdhaqaaqa kakan I iyo soo saarista xad dhaafka ah ee anion Superoxide.Ku-soo-baxa noocyada Oxygen-ka Reactive-ka ah (ROS) ee walxaha wax-soo-saarku waxay u horseedaan hoos u dhac ku yimaada proteolysis autophagic sida lagu muujiyey saamiga LC3-II / LC3-I. habaynta, tarjumaada, laablaabashada borotiinka iyo tamarta tamarta ee mitochondria.Xogtayadu waxay xoojisaa ka qayb qaadashada ururinta ROS ee dabeecadda gabowga ee dhicisoobay waxayna dejinaysaa xidhiidh ka dhexeeya cillad la'aanta mitochondrial iyo luminta boroteostasis ee HGPS.