Prealbumin and Immunoglobulin in Serum and Cerebrospinal Fluid in Familial Amyloid Polyneuropathy

1983 ◽  
Vol 22 (3) ◽  
pp. 213-216 ◽  
Author(s):  
Shin’ichi Shoji ◽  
Noriaki Adachi ◽  
Mitsuaki Kameko ◽  
Shin’ichi Nakagawa
Keyword(s):  
Cerebrospinal Fluid ◽  
Familial Amyloid Polyneuropathy ◽  
Amyloid Polyneuropathy

scholarly journals Transthyretin Misfolding, A Fatal Structural Pathogenesis Mechanism

2021 ◽  
Vol 22 (9) ◽  
pp. 4429
Author(s):  
Jin-Beom Si ◽  
Bokyung Kim ◽  
Jin Hae Kim
Keyword(s):  
Cerebrospinal Fluid ◽  
Thyroid Hormone ◽  
Human Plasma ◽  
Structural Features ◽  
Familial Amyloid Polyneuropathy ◽  
Systemic Amyloidosis ◽  
Retinol Binding Protein ◽  
Senile Systemic Amyloidosis ◽  
Amyloid Polyneuropathy ◽  
Amyloid Cardiomyopathy

Transthyretin (TTR) is an essential transporter of a thyroid hormone and a holo-retinol binding protein, found abundantly in human plasma and cerebrospinal fluid. In addition, this protein is infamous for its amyloidogenic propensity, causing various amyloidoses in humans, such as senile systemic amyloidosis, familial amyloid polyneuropathy, and familial amyloid cardiomyopathy. It has been known for over two decades that decreased stability of the native tetrameric conformation of TTR is the main cause of these diseases. Yet, mechanistic details on the amyloidogenic transformation of TTR were not clear until recent multidisciplinary investigations on various structural states of TTR. In this review, we discuss recent advancements in the structural understanding of TTR misfolding and amyloidosis processes. Special emphasis has been laid on the observations of novel structural features in various amyloidogenic species of TTR. In addition, proteolysis-induced fragmentation of TTR, a recently proposed mechanism facilitating TTR amyloidosis, has been discussed in light of its structural consequences and relevance to acknowledge the amyloidogenicity of TTR.

scholarly journals Clinical and laboratory test in patients with familial amyloid polyneuropathy: differences between symptomatic patients and asymptomatic carriers

2015 ◽  
Vol 10 (S1) ◽  
Author(s):  
Manuel Raya-Cruz ◽  
Juan Buades-Reines ◽  
Cristina Gallego-Lezaun ◽  
Ignacio Ferullo ◽  
Tomas Ripoll-Vera ◽  
...  
Keyword(s):  
Laboratory Test ◽  
Familial Amyloid Polyneuropathy ◽  
Asymptomatic Carriers ◽  
Amyloid Polyneuropathy

scholarly journals Sixty years of transthyretin familial amyloid polyneuropathy (TTR-FAP) in Europe

2016 ◽  
Vol 29 ◽  
pp. S3-S13 ◽  
Author(s):  
Yesim Parman ◽  
David Adams ◽  
Laura Obici ◽  
Lucía Galán ◽  
Velina Guergueltcheva ◽  
...  
Keyword(s):  
Familial Amyloid Polyneuropathy ◽  
Amyloid Polyneuropathy ◽  
Transthyretin Familial Amyloid Polyneuropathy

Electromyographic endings in Italian families with transthyretin (TTR)-Related familial amyloid polyneuropathy (FAP)

1996 ◽  
Vol 6 ◽  
pp. S53
Author(s):  
P. Montagna ◽  
L. Marchello ◽  
R. Plasmati ◽  
R. Vetrugno ◽  
D. Marulli ◽  
...  
Keyword(s):  
Familial Amyloid Polyneuropathy ◽  
Amyloid Polyneuropathy
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