Unusual self-association properties of transthyretin Y114C related to familial amyloidotic polyneuropathy: Effects on detection and quantification

• Tipo: Artigo em Revista Científica Internacional
• Data: 1999

Autores

Ando, Y (Autor) Outra; Almeida, MD (Autor) ICBAS; Ohlsson, PI (Autor) Outra; Ando, E (Autor) Outra; Negi, A (Autor) Outra; Suhr, O (Autor) Outra; Terazaki, H (Autor) Outra; Obayashi, K (Autor) Outra; Ando, M (Autor) Outra; Saraiva, MJM (Autor) ICBAS

Revista

Título: Biochemical and Biophysical Research Communications

Vol. 261

Páginas: 264-269

ISSN: 0006-291X

Editora: Elsevier

Indexação

ISI Web of Knowledge - 11 Citações

Scopus - 10 Citações

Classificação Científica

FOS: Ciências exactas e naturais > Ciências biológicas

Outras Informações

ID Authenticus: P-001-3NK

DOI: 10.1006/bbrc.1999.1048

Abstract (EN): We performed biochemical and immunological examinations of heterozygotic carriers of the transthyretin (TTR) mutant Y114C associated with familial amyloidotic polyneuropathy (FAP), The total serum TTR levels in Y114C TTR carriers were extremely low when analyzed by single radial immunodiffusion (SRID), whereas by indirect enzyme-linked immunosorbent assay (ELISA) procedure, their total TTR concentrations were increased. Recombinant homozygotic Y114C TTR showed no immunoreactivity towards a TTR antibody when analyzed by SRID, whereas by the ELISA procedure presented the same degree of reactivity as that of normal TTR or isolated serum heterozygotic Y114C TTR. These results indicate that immunodifusion based techniques cannot properly determine TTR serum levels in Y114C carriers. Analyses of serum TTR of the Y114C TTR carriers by electrospray ionization mass spectrometry (ESI-MS) with the orifice corn voltage at 60 V revealed a small peak of the free Y114C TTR in addition to large TTR peaks of normal TTR, The levels of the free mutant TTR increased with the orifice corn voltage at 90 V. In contrast, increase in orifice voltage from 60 to 90 V produced a reduction in the level of normal TTR. The results suggest a different pattern of association between monomers in Y114C relative to normal TTR. (C) 1999 Academic Press.

Idioma: Inglês

Tipo (Avaliação Docente): Científica

Nº de páginas: 6