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A Narrative Review of the Role of Transthyretin in Health and Disease

2020Journal article Open access
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dc.contributor.authorLiz, Marcia Almeida
dc.contributor.authorCoelho, Teresa
dc.contributor.authorBellotti, Vittorio
dc.contributor.authorFernandez-Arias, Maria Isabel
dc.contributor.authorMallaina, Pablo
dc.contributor.authorObici, Laura
dc.date.accessioned2022-06-30T10:24:37Z
dc.date.available2022-06-30T10:24:37Z
dc.date.issued2020
dc.description.abstractTransthyretin (TTR) is a tetrameric transport protein highly conserved through vertebrate evolution and synthesized in the liver, choroid plexus, and retinal pigment epithelium. TTR transports the thyroid hormone thyroxine and the retinol-binding protein (RBP) bound to retinol (vitamin A). Mutations in TTR are associated with inherited transthyretin amyloidosis (ATTRv), a progressive, debilitating disease that is ultimately fatal and is characterized by misfolding of TTR and aggregation as amyloid fibrils, predominantly leading to cardiomyopathy or polyneuropathy depending on the particular TTR mutation. Transthyretin amyloid cardiomyopathy can also occur as an age-related disease caused by misfolding of wild-type TTR. Apart from its transport role, little is known about possible additional physiological functions of TTR. Evidence from animal model systems in which TTR has been disrupted via gene knockout is adding to our cumulative understanding of TTR function. There is growing evidence that TTR may have a role in neuroprotection and promotion of neurite outgrowth in response to injury. Here, we review the literature describing potential roles of TTR in neurobiology and in the pathophysiology of diseases other than ATTR amyloidosis. A greater understanding of these processes may also contribute to further clarification of the pathology of ATTR and the effects of potential therapies for TTR-related conditions.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationLiz MA, Coelho T, Bellotti V, Fernandez-Arias MI, Mallaina P, Obici L. A Narrative Review of the Role of Transthyretin in Health and Disease. Neurol Ther. 2020;9(2):395-402. doi:10.1007/s40120-020-00217-0pt_PT
dc.identifier.doi10.1007/s40120-020-00217-0pt_PT
dc.identifier.issn2193-6536
dc.identifier.issn2193-8253
dc.identifier.urihttp://hdl.handle.net/10400.16/2684
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherSpringerpt_PT
dc.relation.publisherversionhttps://link.springer.com/article/10.1007/s40120-020-00217-0pt_PT
dc.rights.urihttp://creativecommons.org/licenses/by-nc/4.0/pt_PT
dc.subjectAlzheimer’s diseasept_PT
dc.subjectAmyloidosispt_PT
dc.subjectNeurodegenerationpt_PT
dc.subjectNeuroprotectionpt_PT
dc.subjectTransthyretinpt_PT
dc.titleA Narrative Review of the Role of Transthyretin in Health and Diseasept_PT
dc.typejournal article
dspace.entity.typePublication
oaire.awardURIinfo:eu-repo/grantAgreement/FCT/Investigador FCT/IF%2F00902%2F2015%2FCP1303%2FCT0001/PT
oaire.citation.conferencePlaceNew Zealandpt_PT
oaire.citation.endPage402pt_PT
oaire.citation.issue2pt_PT
oaire.citation.startPage395pt_PT
oaire.citation.titleNeurology and Therapypt_PT
oaire.citation.volume9pt_PT
oaire.fundingStreamInvestigador FCT
person.familyNameCoelho
person.givenNameTeresa
person.identifier.ciencia-idC71E-3343-F445
project.funder.identifierhttp://doi.org/10.13039/501100001871
project.funder.nameFundação para a Ciência e a Tecnologia
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublicationca69c0a3-d48a-4b92-bbf7-c17288d609b7
relation.isAuthorOfPublication.latestForDiscoveryca69c0a3-d48a-4b92-bbf7-c17288d609b7
relation.isProjectOfPublication6d872f09-973a-4c11-b357-ff3cde183226
relation.isProjectOfPublication.latestForDiscovery6d872f09-973a-4c11-b357-ff3cde183226

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