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Diagnostic and Treatment Approaches Involving Transthyretin in Amyloidogenic Diseases

Transthyretin (TTR) is a thyroid hormone-binding protein which transports thyroxine from the bloodstream to the brain. The structural stability of TTR in tetrameric form is crucial for maintaining its original functions in blood or cerebrospinal fluid (CSF). The altered structure of TTR due to genet...

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Autores principales: Park, Gil Yong, Jamerlan, Angelo, Shim, Kyu Hwan, An, Seong Soo A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6628571/
https://www.ncbi.nlm.nih.gov/pubmed/31216785
http://dx.doi.org/10.3390/ijms20122982
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author Park, Gil Yong
Jamerlan, Angelo
Shim, Kyu Hwan
An, Seong Soo A.
author_facet Park, Gil Yong
Jamerlan, Angelo
Shim, Kyu Hwan
An, Seong Soo A.
author_sort Park, Gil Yong
collection PubMed
description Transthyretin (TTR) is a thyroid hormone-binding protein which transports thyroxine from the bloodstream to the brain. The structural stability of TTR in tetrameric form is crucial for maintaining its original functions in blood or cerebrospinal fluid (CSF). The altered structure of TTR due to genetic mutations or its deposits due to aggregation could cause several deadly diseases such as cardiomyopathy and neuropathy in autonomic, motor, and sensory systems. The early diagnoses for hereditary amyloid TTR with cardiomyopathy (ATTR-CM) and wild-type amyloid TTR (ATTRwt) amyloidosis, which result from amyloid TTR (ATTR) deposition, are difficult to distinguish due to the close similarities of symptoms. Thus, many researchers investigated the role of ATTR as a biomarker, especially its potential for differential diagnosis due to its varying pathogenic involvement in hereditary ATTR-CM and ATTRwt amyloidosis. As a result, the detection of ATTR became valuable in the diagnosis and determination of the best course of treatment for ATTR amyloidoses. Assessing the extent of ATTR deposition and genetic analysis could help in determining disease progression, and thus survival rate could be improved following the determination of the appropriate course of treatment for the patient. Here, the perspectives of ATTR in various diseases were presented.
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spelling pubmed-66285712019-08-05 Diagnostic and Treatment Approaches Involving Transthyretin in Amyloidogenic Diseases Park, Gil Yong Jamerlan, Angelo Shim, Kyu Hwan An, Seong Soo A. Int J Mol Sci Review Transthyretin (TTR) is a thyroid hormone-binding protein which transports thyroxine from the bloodstream to the brain. The structural stability of TTR in tetrameric form is crucial for maintaining its original functions in blood or cerebrospinal fluid (CSF). The altered structure of TTR due to genetic mutations or its deposits due to aggregation could cause several deadly diseases such as cardiomyopathy and neuropathy in autonomic, motor, and sensory systems. The early diagnoses for hereditary amyloid TTR with cardiomyopathy (ATTR-CM) and wild-type amyloid TTR (ATTRwt) amyloidosis, which result from amyloid TTR (ATTR) deposition, are difficult to distinguish due to the close similarities of symptoms. Thus, many researchers investigated the role of ATTR as a biomarker, especially its potential for differential diagnosis due to its varying pathogenic involvement in hereditary ATTR-CM and ATTRwt amyloidosis. As a result, the detection of ATTR became valuable in the diagnosis and determination of the best course of treatment for ATTR amyloidoses. Assessing the extent of ATTR deposition and genetic analysis could help in determining disease progression, and thus survival rate could be improved following the determination of the appropriate course of treatment for the patient. Here, the perspectives of ATTR in various diseases were presented. MDPI 2019-06-18 /pmc/articles/PMC6628571/ /pubmed/31216785 http://dx.doi.org/10.3390/ijms20122982 Text en © 2019 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Park, Gil Yong
Jamerlan, Angelo
Shim, Kyu Hwan
An, Seong Soo A.
Diagnostic and Treatment Approaches Involving Transthyretin in Amyloidogenic Diseases
title Diagnostic and Treatment Approaches Involving Transthyretin in Amyloidogenic Diseases
title_full Diagnostic and Treatment Approaches Involving Transthyretin in Amyloidogenic Diseases
title_fullStr Diagnostic and Treatment Approaches Involving Transthyretin in Amyloidogenic Diseases
title_full_unstemmed Diagnostic and Treatment Approaches Involving Transthyretin in Amyloidogenic Diseases
title_short Diagnostic and Treatment Approaches Involving Transthyretin in Amyloidogenic Diseases
title_sort diagnostic and treatment approaches involving transthyretin in amyloidogenic diseases
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6628571/
https://www.ncbi.nlm.nih.gov/pubmed/31216785
http://dx.doi.org/10.3390/ijms20122982
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