원문정보
초록
영어
Gangliosides has the important roles in interactions between cells and in signal transduction to regulate growth and differentiation. Hutchinson-Gilford progeria syndrome (HGPS) is an extremely rare genetic disorder wherein symptoms resembling aspects of aging are manifested at a very early age. Model of LMNA (Lamin A/C) mutation is known as a typical model for HPGS. However, roles of gangliosides in model of LMNA mutation (HGPS) is unclear. Therefore, this study investigated the expression patterns of gangliosides in model of LMNA-mutation. LMNA-mutation cells were primary cultured from spontaneous mouse LMNA-mutation model. Distribution of gangliosides in LMNA-mutation mouse was detected by HPTLC. We successes the primary cultured bone marrow-derived mesencymal stem cells from LMNA-mutation mouse. In addition, gangliosides in several tissues including liver, kidney and brain from LMNA-mutation mouse was various expressed compare with normal tissues. So, gangliosides may be related with LMNA in cell differentiation and proliferation. Therefore, this results suggested that gangliosides act important roles in aging and HGPS.