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Int. J. Mol. Sci. 2018, 19(1), 320; doi:10.3390/ijms19010320

Hereditary Fibrinogen Aα-Chain Amyloidosis in Asia: Clinical and Molecular Characteristics

1
Institute for Biomedical Sciences, Shinshu University, Matsumoto 390-8621, Japan
2
Department of Clinical Laboratory Medicine, Shinshu University School of Health Sciences, Matsumoto 390-8621, Japan
3
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto 390-8621, Japan
4
Tokyo Metropolitan Institute of Medical Science, Tokyo 156-8506, Japan
*
Author to whom correspondence should be addressed.
Received: 26 December 2017 / Revised: 18 January 2018 / Accepted: 19 January 2018 / Published: 22 January 2018
(This article belongs to the Special Issue Genetic Basis of Fibrinogen Disorders)
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Abstract

Hereditary fibrinogen Aα-chain amyloidosis (Aα-chain amyloidosis) is a type of autosomal dominant systemic amyloidosis caused by mutations in fibrinogen Aα-chain gene (FGA). Patients with Aα-chain amyloidosis have been mainly reported in Western countries but have been rarely reported in Asia, with only five patients with Aα-chain amyloidosis being reported in Korea, China, and Japan. Clinically, the most prominent manifestation in Asian patients with Aα-chain amyloidosis is progressive nephropathy caused by excessive amyloid deposition in the glomeruli, which is similar to that observed in patients with Aα-chain amyloidosis in Western countries. In molecular features in Asian Aα-chain amyloidosis, the most common variant, E526V, was found in only one Chinese kindred, and other four kindred each had a different variant, which have not been identified in other countries. These variants are located in the C-terminal region (amino acid residues 517–555) of mature Aα-chain, which was similar to that observed in patients with Aα-chain amyloidosis in other countries. The precise number of Asian patients with Aα-chain amyloidosis is unclear. However, patients with Aα-chain amyloidosis do exist in Asian countries, and the majority of these patients may be diagnosed with other types of systemic amyloidosis. View Full-Text
Keywords: fibrinogen Aα-chain amyloidosis; hereditary renal amyloidosis; Ostertag-type amyloidosis; laser microdissection; proteomics analysis fibrinogen Aα-chain amyloidosis; hereditary renal amyloidosis; Ostertag-type amyloidosis; laser microdissection; proteomics analysis
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Yazaki, M.; Yoshinaga, T.; Sekijima, Y.; Kametani, F.; Okumura, N. Hereditary Fibrinogen Aα-Chain Amyloidosis in Asia: Clinical and Molecular Characteristics. Int. J. Mol. Sci. 2018, 19, 320.

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