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การแสดงออกของโปรตีน Heat Shock 60 (Hsp60) ในไมโทคอนเดรียของผู้ป่วยโรค Leber Hereditary Optic Neuropathy ชาวไทย (The Expression of Mitochondrial Heat Shock Protein 60 (Hsp60) in Thai Leber Hereditary Optic Neuropathy Patients) |
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| รหัสดีโอไอ | |
| Creator | Pratibha Aryal |
| Title | การแสดงออกของโปรตีน Heat Shock 60 (Hsp60) ในไมโทคอนเดรียของผู้ป่วยโรค Leber Hereditary Optic Neuropathy ชาวไทย (The Expression of Mitochondrial Heat Shock Protein 60 (Hsp60) in Thai Leber Hereditary Optic Neuropathy Patients) |
| Publisher | Genetics Society of Thailand |
| Publication Year | 2556 |
| Journal Title | Thai Journal of Genetics |
| Journal Vol. | S.1 |
| Journal No. | S1 (Special Issue 1) |
| Page no. | 91 |
| Keyword | LHON, mitochondrial protein, Hsp60 |
| ISSN | 8578664 |
| Abstract | Leber Hereditary Optic Neuropathy (LHON) is one of the most common mitochondrial diseases that can cause complete blindness, particularly in young men. The majority of LHON cases are caused by one of the three prevalent mitochondrial DNA (mtDNA) mutations -G3460A/ND1, G11778A/ND4, and T14484C/ND6. However these mutations cannot explain the several remarkable features of LHON, therefore, the nuclear factors is suggested to be involved in the phenotypic expression of LHON. Our Previous findings demonstrated 29 nuclear-encoded mitochondrial proteins with altered expression pattern between affected and unaffected groups among Thai LHON patients. In the present study, heat shock protein 60 (Hsp60) a vital mitochondrial chaperone was investigated and found to be significantly down regulated in 11778G>A LHON affected fibroblast compared to the fibroblasts from either control individuals or from unaffected relatives. Therefore, the reduced expression level of Hsp60 in the fibroblast of affected LHON groups may suggest the defect in the protein turnover of the mitochondria, whichmight contribute to the pathogenesis of LHON. |
