Changes in vimentin, lamin A/C and mitofilin induce?aberrant cell organization in fibroblasts from Fanconi anemia complementation group A (FA-A) patients

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• 作者：Cristina Capanni ; Maurizio Bruschi ; Marta Columbaro ; Paola Cuccarolo ; Silvia Ravera ; Carlo Dufour ; Giovanni C ; iano ; Andrea Petretto ; Paolo Degan ; Enrico Cappelli
• 关键词：Fanconi anemia ; Oxidative stress ; Cell structure
• 刊名：Biochimie
• 出版年：2013
• 出版时间：October, 2013
• 年：2013
• 卷：95
• 期：10
• 页码：1838-1847
• 全文大小：2354 K

文摘

Growing number of publication has proved an increasing of cellular function of the Fanconi anemia proteins. To chromosome stability and DNA repair new roles have been attributed to FA proteins in oxidative stress response and homeostasis, immune response and cytokines sensibility, gene expression. Our work shows a new role for FA-A protein: the organization of the cellular structure.

By 2D-PAGE of FA-A and correct fibroblasts treated and untreated with H₂O₂ we identify different expression of protein involved in the structural organization of nucleus, intermediate filaments and mitochondria. Immunofluorescence and electronic microscopy analysis clearly show an already altered cellular structure in normal culture condition and this worsted after oxidative stress. FA-A cell appears structurally prone to physiologic stress and this could explain part of the phenotype of FA cells.