临床与实验病理学杂志2011，Vol.27Issue(7)：739-741,745,4.
FAP患者心脏组织淀粉样变形成机制分析
Analysis of amyloid formation mechanism in heart tissues of FAP patients
摘要
Abstract
Purpose To investigate amyloid formation mechanism of mutated transthyretin( TTR ) in familial amyloidotic polyneuropathy ( FAP ). Methods ( 1 ) Matrix assisted laser desorption ionisation time-of-flight mass spectrometry ( MALDI-TOF/MS ) was used to determine the gene mutation type of TTR. ( 2 ) Amyloid deposition in heart tissues was confirmed by Congo red stain and polarized light microscopy. ( 3 ) Amyloid preprotein type was determined by immunohistochemistry. ( 4 ) Amyloid deposition extent and morphological characteristics were analyzed with histological morphology analysis. Results ( 1 ) The gene mutation type was ATTRVal30Met as shown by results of serum TTR analysis with MALDI-TOF/MS. ( 2 ) Amyloid was found in small blood vessels and myocardial interstitial in heart tissues, as confirmed by Congo red stain and apple green birefringence under polarized light microscopy. ( 3 ) The amyloid preprotein was demonstrated to be TTR by immunohistochemistry. ( 4 ) Congo red stain extent in loose tissues between vascular walls and myocardial fibers was significantly higher than that of other parts of heart tissues. Conclusions Amyloid deposition is significant in myocardial interstitial and loose connective tissues of FAP ATTRVal30Met patients, suggesting that factors such as carrier transportation and chemical modification may play important roles in TTR amyloidosis.关键词
心肌淀粉样变/家族性淀粉样变多发性神经损害/刚果红/转甲状腺素蛋白Key words
cardiac muscle amvloid/ familial amyloidotic polyneuropathy/ Congo red/ transthyretin分类
医药卫生引用本文复制引用
宋林,Mitsuharu Ueda,段红茹,马玉株,倪敏,孙续国,Yukio Ando..FAP患者心脏组织淀粉样变形成机制分析[J].临床与实验病理学杂志,2011,27(7):739-741,745,4.基金项目
国家自然科学基金资助(30973157) （30973157）
