Evidence of Oxidative Stress in Familial Amyloidotic Polyneuropathy Type 1

Abstract

Oxidative stress may be part of the mechanisms leading to tissue damage in patients with FAP1. The lack of correlation between the laboratory findings and the severity of clinical involvement may signal that oxidative processes are at work throughout the natural history of the disease.

Small Transthyretin (TTR) Ligands as Possible Therapeutic Agents in TTR Amyloidoses

“…Similar studies using neuroblastomas revealed also apoptotic effects in neuroblastomas [52]. In addition, oxidative stress has been reported in FAP patients [53,54]. Therefore free radical scavengers seemed to be candidates to be used as therapeutic agents in FAP, inhibiting the deleterious effects of TTR aggregates.…”

Small Transthyretin (TTR) Ligands as Possible Therapeutic Agents in TTR Amyloidoses

“…Similar studies using neuroblastomas revealed also apoptotic effects in neuroblastomas [52]. In addition, oxidative stress has been reported in FAP patients [53,54]. Therefore free radical scavengers seemed to be candidates to be used as therapeutic agents in FAP, inhibiting the deleterious effects of TTR aggregates.…”

Neuroprotective effect against amyloidogenic transthyretin aggregates - Induced cytotoxicity on human neuroblastoma cell by phenolic-rich Centella asiatica extract

“…Although a complete picture of the precise molecular mechanisms underlying TTR aggregate mediated tissue damage is still emerging, what is undisputed is the fact that early events of this process is preceded by deposition of the amyloidogenic nonfibrillar TTR aggregates [ 55 ]. The involvement of oxidative stress occasioned by deposition of transthyretin amyloid species both at the cellular and tissue levels has been well documented [ 5 , 6 , 50 , 51 , 56 ]. Interestingly, oxidation has also been implicated in promoting the process of transthyretin aggregation [ 57 , 58 ], and thereby facilitate the adverse cellular effects triggered by the protein amyloid aggregates.…”

Anterior Chamber Flare as an Objective and Quantitative Noninvasive Method for Oculopathy in Transthyretin V30M Amyloidosis Patients

“…The damage should be from outside the vessels were circulates the mutant TTR (aqueous humor and vitreous). As ATTR V30M patients have a proinflammatory state [ 35 ], we hypothesize that in response to this stimulation, the ciliary pigmented epithelium may release a large variety of cytokines and fibrin aggregates which may induce the breakdown of the BAB and outflow resistance in the anterior segment of the eye in ATTRV30M patients. As described previously, the intraocular production of TTR V30M by the retinal and ciliary pigment epithelium remains unchanged in these patients [ 8 ].…”

Small Transthyretin (TTR) Ligands as Possible Therapeutic Agents in TTR Amyloidoses

“…Similar studies using neuroblastomas revealed also apoptotic effects in neuroblastomas [52]. In addition, oxidative stress has been reported in FAP patients [53,54]. Therefore free radical scavengers seemed to be candidates to be used as therapeutic agents in FAP, inhibiting the deleterious effects of TTR aggregates.…”

Neuroprotective effect against amyloidogenic transthyretin aggregates - Induced cytotoxicity on human neuroblastoma cell by phenolic-rich Centella asiatica extract

“…Although a complete picture of the precise molecular mechanisms underlying TTR aggregate mediated tissue damage is still emerging, what is undisputed is the fact that early events of this process is preceded by deposition of the amyloidogenic nonfibrillar TTR aggregates [ 55 ]. The involvement of oxidative stress occasioned by deposition of transthyretin amyloid species both at the cellular and tissue levels has been well documented [ 5 , 6 , 50 , 51 , 56 ]. Interestingly, oxidation has also been implicated in promoting the process of transthyretin aggregation [ 57 , 58 ], and thereby facilitate the adverse cellular effects triggered by the protein amyloid aggregates.…”

Anterior Chamber Flare as an Objective and Quantitative Noninvasive Method for Oculopathy in Transthyretin V30M Amyloidosis Patients

“…The damage should be from outside the vessels were circulates the mutant TTR (aqueous humor and vitreous). As ATTR V30M patients have a proinflammatory state [ 35 ], we hypothesize that in response to this stimulation, the ciliary pigmented epithelium may release a large variety of cytokines and fibrin aggregates which may induce the breakdown of the BAB and outflow resistance in the anterior segment of the eye in ATTRV30M patients. As described previously, the intraocular production of TTR V30M by the retinal and ciliary pigment epithelium remains unchanged in these patients [ 8 ].…”

Small Transthyretin (TTR) Ligands as Possible Therapeutic Agents in TTR Amyloidoses

“…Similar studies using neuroblastomas revealed also apoptotic effects in neuroblastomas [52]. In addition, oxidative stress has been reported in FAP patients [53,54]. Therefore free radical scavengers seemed to be candidates to be used as therapeutic agents in FAP, inhibiting the deleterious effects of TTR aggregates.…”

Neuroprotective effect against amyloidogenic transthyretin aggregates - Induced cytotoxicity on human neuroblastoma cell by phenolic-rich Centella asiatica extract

“…Although a complete picture of the precise molecular mechanisms underlying TTR aggregate mediated tissue damage is still emerging, what is undisputed is the fact that early events of this process is preceded by deposition of the amyloidogenic nonfibrillar TTR aggregates [ 55 ]. The involvement of oxidative stress occasioned by deposition of transthyretin amyloid species both at the cellular and tissue levels has been well documented [ 5 , 6 , 50 , 51 , 56 ]. Interestingly, oxidation has also been implicated in promoting the process of transthyretin aggregation [ 57 , 58 ], and thereby facilitate the adverse cellular effects triggered by the protein amyloid aggregates.…”

Anterior Chamber Flare as an Objective and Quantitative Noninvasive Method for Oculopathy in Transthyretin V30M Amyloidosis Patients

“…The damage should be from outside the vessels were circulates the mutant TTR (aqueous humor and vitreous). As ATTR V30M patients have a proinflammatory state [ 35 ], we hypothesize that in response to this stimulation, the ciliary pigmented epithelium may release a large variety of cytokines and fibrin aggregates which may induce the breakdown of the BAB and outflow resistance in the anterior segment of the eye in ATTRV30M patients. As described previously, the intraocular production of TTR V30M by the retinal and ciliary pigment epithelium remains unchanged in these patients [ 8 ].…”