5 documents found, page 1 of 1
The Expression of Chemokines Is Downregulated in a Pre-Clinical Model of TTR V3...
Moreira, J; Costelha, S; Saraiva, M; Saraiva, MJMInflammation is a hallmark of several neurodegenerative disorders including hereditary amyloidogenic transthyretin amyloidosis (ATTRv). ATTRv is an autosomal dominant neurodegenerative disorder with extracellular deposition of mutant transthyretin (TTR) aggregates and fibrils, particularly in nerves and ganglia of the peripheral nervous system. Nerve biopsies from ATTRv patients show increased cytokine producti...
In vitro and in vivo effects of SerpinA1 on the modulation of transthyretin pro...
Bezerra, F; Niemietz, C; Schmidt, HHJ; Zibert, A; Guo, S; Monia, BP; Gonçalves, P; Saraiva, MJM; Almeida, MRTransthyretin (TTR) proteolysis has been recognized as a complementary mechanism contributing to transthyretin-related amyloidosis (ATTR amyloidosis). Accordingly, amyloid deposits can be composed mainly of full-length TTR or contain a mixture of both cleaved and full-length TTR, particularly in the heart. The fragmentation pattern at Lys48 suggests the involvement of a serine protease, such as plasmin. The mos...
A human antibody selective for transthyretin amyloid removes cardiac amyloid th...
Michalon, A; Hagenbuch, A; Huy, C; Varela, E; Combaluzier, B; Damy, T; Suhr, OB; Saraiva, MJM; Hock, C; Nitsch, RM; Grimm, JTransthyretin amyloid (ATTR) cardiomyopathy is a debilitating disease leading to heart failure and death. It is characterized by the deposition of extracellular ATTR fibrils in the myocardium. Reducing myocardial ATTR load is a therapeutic goal anticipated to translate into restored cardiac function and improved patient survival. For this purpose, we developed the selective anti-ATTR antibody NI301A, a recombin...
Modulation of the Mechanisms Driving Transthyretin Amyloidosis
Bezerra, F; Saraiva, MJM; Almeida, MRTransthyretin (TTR) amyloidoses are systemic diseases associated with TTR aggregation and extracellular deposition in tissues as amyloid. The most frequent and severe forms of the disease are hereditary and associated with amino acid substitutions in the protein due to single point mutations in the TTR gene (ATTRv amyloidosis). However, the wild type TTR (TTR wt) has an intrinsic amyloidogenic potential that, i...
Uncovering the neuroprotective mechanisms of curcumin on transthyretin amyloidosis
Ferreira, N; Saraiva, MJM; Almeida, MRTransthyretin (TTR) amyloidoses (ATTR amyloidosis) are diseases associated with transthyretin (TTR) misfolding, aggregation and extracellular deposition in tissues as amyloid. Clinical manifestations of the disease are variable and include mainly polyneuropathy and/or cardiomyopathy. The reasons why TTR forms aggregates and amyloid are related with amino acid substitutions in the protein due to mutations, or wi...