Ngwaahịa

Nchịkọta nuklia nke ụdị mutant nke protein Lamin-A, nke a na-akpọ Progerin (PG) ma ọ bụ Lamin AΔ50, na-eme na Hutchinson-Gilford Progeria Syndrome (HGPS) ma ọ bụ Progeria, ọrịa ịka nká na-arịwanye elu.Otu n'ime isi ihe mgbaàmà nke ọrịa mkpụrụ ndụ ihe nketa a bụ mfu nke abụba subcutaneous n'ihi lipodystrophy dị egwu. Anyị kpalitere okwu PG mmadụ na GFP -Green Fluorescent Protein - dị ka njikwa na sel 3T3L1 na-eji usoro lentiviral mụọ mmetụta nke okwu PG na ikike dị iche iche nke ahịrị cell a, otu n'ime ụdị adipogenic kachasị eji.A na-eme proteomics quantitative (iTRAQ) iji mụọ mmetụta nke mkpokọta PG.A kwadoro ọtụtụ n'ime protein ndị a gbanwere site na immunoblotting na PCR dị adị.A na-enyocha ọrụ Mitochondrial site na nha nke a) ọrụ mitochondrial basal, b) mmepụta nke superoxide anion na c) arụmọrụ nke onye ọ bụla nke mgbagwoju anya dị iche iche nke usoro iku ume. Anyị chọpụtara na PG n'elu-egosipụta site na lentivi ral gene nnyefe na-eduga na a mbelata n'ọkwa ụba na ntụpọ na ikike adipogenic ma e jiri ya tụnyere njikwa.Nyocha ọnụọgụ proteomics gosipụtara protein 181 nke ọma (p<0.05) emezigharịrị na PG-egosipụta preadipocytes.Ọrụ Mitochondrial adịghị arụ ọrụ na sel na-egosipụta PG.Kpọmkwem, anyị achọpụtala mmụba nke ọrụ mgbagwoju anya nke m yana mmepụta nke Superoxide anion.Incubation na Reactive Oxygen Species (ROS) scavenger agents na-eme ka mbelata nke proteolysis autophagic dị ka LC3-II/LC3-I ratio gosipụtara.PG okwu na sel 3T3L1 na-akwalite mgbanwe n'ọtụtụ usoro ndu, gụnyere nhazi nke cytoskeleton, metabolism lipid, calcium. ụkpụrụ, ntụgharị asụsụ, mpịakọta protein na ike site na mitochondria.Data anyị na-ewusi ntinye aka nke mkpokọta ROS na ụdị ịka nká phenotype ma guzobe njikọ n'etiti arụ ọrụ mitochondrial na ọnwụ nke proteostasis na HGPS.