Transthyretin-Related Familial Amyloidotic Polyneuropathy

Epidemiology of familial amyloid polyneuropathy in Japan: Identification of a novel endemic focus

“…High prevalence in Nagano and Kumamoto prefectures, which presented with major endemic foci of FAP, were consistent with the previous reports [4,7,8,11,16]. Notably, our study revealed that Ishikawa prefecture is the third endemic focus of FAP in Japan.…”

Epidemiology of familial amyloid polyneuropathy in Japan: Identification of a novel endemic focus

“…High prevalence in Nagano and Kumamoto prefectures, which presented with major endemic foci of FAP, were consistent with the previous reports [4,7,8,11,16]. Notably, our study revealed that Ishikawa prefecture is the third endemic focus of FAP in Japan.…”

Long-term survival after liver transplantation in patients with familial amyloid polyneuropathy

“…7 The survival period of these patients was similar to that reported previously. The nontransplant group in this study comprised patients with ordinary-onset FAP Val30Met that followed the natural history of the disease.…”

S-Homocysteinylation effects on transthyretin: worsening of cardiomyopathy onset

“…Recent data have reported that the thiol group of Cys 10 found in monomeric TTR is remarkably reactive and that its oxidation is involved in TTR tetramer destabilization [39,40]. These findings agree with the severe oxidative stress observed in FAP patients [14]. In addition to oxidation, other post-translational modification modify tetramer stability.…”

Epidemiology of familial amyloid polyneuropathy in Japan: Identification of a novel endemic focus

“…High prevalence in Nagano and Kumamoto prefectures, which presented with major endemic foci of FAP, were consistent with the previous reports [4,7,8,11,16]. Notably, our study revealed that Ishikawa prefecture is the third endemic focus of FAP in Japan.…”

Long-term survival after liver transplantation in patients with familial amyloid polyneuropathy

“…7 The survival period of these patients was similar to that reported previously. The nontransplant group in this study comprised patients with ordinary-onset FAP Val30Met that followed the natural history of the disease.…”

S-Homocysteinylation effects on transthyretin: worsening of cardiomyopathy onset

“…Recent data have reported that the thiol group of Cys 10 found in monomeric TTR is remarkably reactive and that its oxidation is involved in TTR tetramer destabilization [39,40]. These findings agree with the severe oxidative stress observed in FAP patients [14]. In addition to oxidation, other post-translational modification modify tetramer stability.…”

Epidemiology of familial amyloid polyneuropathy in Japan: Identification of a novel endemic focus

“…High prevalence in Nagano and Kumamoto prefectures, which presented with major endemic foci of FAP, were consistent with the previous reports [4,7,8,11,16]. Notably, our study revealed that Ishikawa prefecture is the third endemic focus of FAP in Japan.…”

Long-term survival after liver transplantation in patients with familial amyloid polyneuropathy

“…7 The survival period of these patients was similar to that reported previously. The nontransplant group in this study comprised patients with ordinary-onset FAP Val30Met that followed the natural history of the disease.…”

S-Homocysteinylation effects on transthyretin: worsening of cardiomyopathy onset

“…Recent data have reported that the thiol group of Cys 10 found in monomeric TTR is remarkably reactive and that its oxidation is involved in TTR tetramer destabilization [39,40]. These findings agree with the severe oxidative stress observed in FAP patients [14]. In addition to oxidation, other post-translational modification modify tetramer stability.…”