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Titolo:
Vitreous amyloidosis after liver transplantation in patients with familialamyloid polyneuropathy: ocular synthesis of mutant transthyretin
Autore:
Munar-Ques, M; Salva-Ladaria, L; Mulet-Perera, P; Sole, M; Lopez-Andreu, FR; Saraiva, MJM;
Indirizzi:
PAF, Grp Estudio, Palma de Mallorca, Spain PAF Palma de Mallorca SpainPAF, Grp Estudio, Palma de Mallorca, Spain Hosp Clin Barcelona, Serv Anat Patol, Barcelona, Spain Hosp Clin Barcelona Barcelona Spain , Serv Anat Patol, Barcelona, Spain Hosp Gen Univ, Murcia, Spain Hosp Gen Univ Murcia SpainHosp Gen Univ, Murcia, Spain Univ Porto, Inst Mol & Cellular Biol, Amyloid Unit, P-4100 Porto, PortugalUniv Porto Porto Portugal P-4100 l, Amyloid Unit, P-4100 Porto, Portugal
Titolo Testata:
AMYLOID-INTERNATIONAL JOURNAL OF EXPERIMENTAL AND CLINICAL INVESTIGATION
fascicolo: 4, volume: 7, anno: 2000,
pagine: 266 - 269
SICI:
1350-6129(200012)7:4<266:VAALTI>2.0.ZU;2-2
Fonte:
ISI
Lingua:
ENG
Soggetto:
PROTEIN; BRAIN;
Keywords:
amyloid; familial amyloidotic polyneuropathy; transthyretin; vitreous; liver transplantation;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
14
Recensione:
Indirizzi per estratti:
Indirizzo: Saraiva, MJM Univ Porto, Inst Mol & Cellular Biol, Amyloid Unit, Rua CampoAlegre 823, P-4100 Porto, Portugal Univ Porto Rua Campo Alegre 823 Porto Portugal P-4100 rtugal
Citazione:
M. Munar-Ques et al., "Vitreous amyloidosis after liver transplantation in patients with familialamyloid polyneuropathy: ocular synthesis of mutant transthyretin", AMYLOID, 7(4), 2000, pp. 266-269

Abstract

Vitreous amyloidosis has bee reported in patients with familial amyloidotic polyneuropathy (FAP) who are carriers of different mutant transthyretins (TTR). The mutant TTR constitutes the majority of the amyloid vitreous fibrils in heterozygous Val30Met patients. Due to the ocular synthesis of TTR, it is possible that the retina constitutes the source of vitreous amyloid fibrils; if so, orthotopic liver transplantation (OLT) performed to remove the mutant TTR from circulation might not be effective in treating/avoiding vitreous amyloid. We present vitreous amyloidosis in a FAB patient from Maiorca with ATTR Val30Met who underwent OLT at age 38. Progressive impairmentof visual acuity (VA) appeared bilaterally 2 years after OLT due to vitreous opacities consistent with amyloid; successful bilateral vitrectomy was performed. Amyloid was demonstrated in the vitrectomy material by Congo red staining; immunohistochemistry and Western blotting analyses were positive with an antibody for human TTR. Mass spectrometry of TTR revealed the presence of the mutant in approximately 20% of the TTR. Future structural studies on vitreous material with different proportions of normal/versus mutant TTR might shed some light on TTR fibrillogenesis. These results show that vitreous deposition of TTR amyloid fibrils occurs after OLT, suggesting that ongoing intraocular synthesis of mutant TTR might contribute to this process. We also present the progression after OLT of vitreous amyloidosis previously diagnosed in three patients with TTR Val71Ala.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 04/04/20 alle ore 14:21:34