肝移植后转甲状腺素蛋白淀粉样变性神经病的进展。
Progression of transthyretin amyloid neuropathy after liver transplantation.
机构信息
Indiana University School of Medicine, Department of Pathology and Laboratory Medicine, 635 Barnhill Drive, Indianapolis, IN 46202-5126, USA.
出版信息
Neurology. 2010 Jul 27;75(4):324-7. doi: 10.1212/WNL.0b013e3181ea15d4.
OBJECTIVE
To biochemically characterize peripheral nerve amyloid in subjects with transthyretin (TTR) amyloidosis and assess effect of orthotopic liver transplantation (OLT) on progression of neuropathy.
METHODS
Amyloid fibrils were isolated from peripheral nerve tissues of 6 patients with TTR amyloidosis who were heterozygous for an amyloid-associated TTR mutation. Ratio of variant to wild-type TTR in the fibrils was determined by amino acid sequencing of tryptic peptides containing either the variant amino acid residue or the corresponding normal amino acid.
RESULTS
Amyloid fibrils from 3 subjects who died without having received a liver transplant were composed of 60%-65% variant TTR and 35%-40% wild-type. Amyloid fibrils from a subject who died 5 years after liver transplantation contained 25% variant and 75% wild-type TTR.
CONCLUSION
Ratios of variant to wild-type TTR in amyloid patients heterozygous for an amyloid-associated TTR mutation are similar to published ratios for amyloid fibrils in cardiac tissue. Survival after liver transplantation for TTR amyloidosis may be associated with progression of neuropathy due to continued deposition of amyloid derived from wild-type TTR.
目的
对转甲状腺素蛋白（TTR）淀粉样变患者的周围神经淀粉样变进行生化分析，并评估原位肝移植（OLT）对神经病变进展的影响。
方法
从 6 名 TTR 淀粉样变的杂合子患者的周围神经组织中分离出淀粉样纤维。通过含有变异氨基酸残基或相应正常氨基酸的胰蛋白酶肽的氨基酸序列测定，确定纤维中变异型与野生型 TTR 的比例。
结果
3 名未接受肝移植而死亡的患者的淀粉样纤维由 60%-65%的变异型 TTR 和 35%-40%的野生型 TTR 组成。肝移植 5 年后死亡的患者的淀粉样纤维中含有 25%的变异型和 75%的野生型 TTR。
结论
杂合子 TTR 淀粉样变患者的淀粉样纤维中变异型与野生型 TTR 的比例与心脏组织中报道的淀粉样纤维的比例相似。TTR 淀粉样变性患者肝移植后的存活可能与源自野生型 TTR 的淀粉样沉积导致的神经病变进展有关。
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