Protease and Protease Inhibitors in Familial Amyloidotic Polyneuropathy
The concentration of six protease inhibitors were studied in patients with familial amyloidotic polyneuropathy (FAP) and in healthy controls, and no significant differences were detected. The proteolytic effect of brinase, an enzyme of Aspergillus oryza, on amyloid tissue sections from patients with FAP was also evaluated. Amyloid fibrils were degraded by brinase, while the tissue structures remained fairly intact.
KeywordsProtease Inhibitor Amyloid Fibril Aspergillus Oryzae Secondary Amyloidosis Familial Amyloidotic Polyneuropathy
Unable to display preview. Download preview PDF.
- Adachi N., S Shoji, N Yanagisawa (in press) Bleeding manifestations in 24 patients with familial amyloidotic polyneuropathy. Europ NeurolGoogle Scholar
- Eriksson, S. (1960) Determination of serum trypsin inhibitor capacity. Acta Med. Scand., 432(suppl.):6–85Google Scholar
- Kato H, N Adachi, S Iwanaga, K Abe, K Takada, T Kimura, S Sakakibara (1980) A fluorogenic substrate method for the estimation of kallikrein in urine. J Biochem. 87:1127–1132Google Scholar
- Kato H, N Adachi, Y Ohno, S Iwanaga, K Takada and S Sakakibara (1980) New fluorogenic peptide substrates for plasmin. J Biochem 88:183–190Google Scholar
- Kedar I., M. Ravid and E. Sohar (1974) Demonstration of amyloid degrading activity in normal human serum. Proc. Soc. Exp. Biol. Med., 145: 343–345Google Scholar
- Kedar I, Sohar E, Ravid M. (1982) Degradation of amyloid by a serum component and inhibition of degradation. J Lab Clin Med 99:693–700Google Scholar
- Saraiva, M.J.M., P.P. Costa and D.S. Goodman (1986) Genetic expression of a transthyretin mutation in typical and late-onset Portugese families with familial amyloidotic polyneuropathy. Neurology, 36: 1423–1417Google Scholar