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Glossary
ATTR-CM – Transthyretin amyloid cardiomyopathy. ATTR-CM is a progressive condition that mimics heart disease. It is caused when the protein transthyretin (TTR) destabilizes and misfolds into toxic amyloid fibrils that accumulate in the myocardium.1,2
ATTRv-CM – Variant (also known as hereditary, ATTRv-CM) transthyretin amyloid cardiomyopathy. A form of ATTR-CM caused by destabilized amino acid variants in the transthyretin (TTR) protein, which leads to the development of amyloid fibrils.1
ATTRwt-CM – Wild-type transthyretin amyloid cardiomyopathy. A form of ATTR-CM in which spontaneous destabilization of the transthyretin protein occurs due to aging.1
NT-proBNP – N-terminal pro-B-type natriuretic peptide. A protein secreted by the heart in response to stress. Elevated levels of NT-proBNP have been associated with an increased risk for mortality and hospitalization in patients with ATTR-CM. Lower levels of NT-proBNP indicate better cardiac function.3-5
T119M – A naturally occurring, superstabilizing variant of the transthyretin protein shown to protect against severe manifestations of ATTR.6,7
TTR – Transthyretin, also known as prealbumin. A key transport protein for retinol binding protein (vitamin A) and thyroxine (thyroid hormone) in the blood. TTR is primarily produced in the liver and adopts a tetrameric structure to transport cargo through the body.8,9
V122I – One of the most common pathogenic variants of the transthyretin protein, occurring in 3%-4% of Black Americans. This variant has been associated with severe congestive heart failure.10
Kittleson MM, et al. Cardiac Amyloidosis: Evolving Diagnosis and Management: A Scientific Statement from the American Heart Association. Circulation. 2020;142(1):e7-e22. 2. Garcia-Pavia P, et al. Expert Consensus on the Monitoring of Transthyretin Amyloid Cardiomyopathy. Eur J Heart Fail. 2021;23(6):895-905. 3. Oghina S, et al. Prognostic Value of N-Terminal Pro-Brain Natriuretic Peptide and High-Sensitivity Troponin T Levels in the Natural History of Transthyretin Amyloid Cardiomyopathy and Their Evolution After Tafamidis Treatment. J Clin Med. 2021;10(21):4868. 4. Law S, et al. Change in N-terminal Pro-B-Type Natriuretic Peptide at 1 Year Predicts Mortality in Wild-Type Transthyretin Amyloid Cardiomyopathy. Heart. 2022;108(6):474-478. 5. Mueller C, et al. Heart Failure Association of the European Society of Cardiology Practical Guidance on the Use of Natriuretic Peptide Concentrations. Eur J Heart Fail. 2019;21(6):715-731. 6. Hammarström P, et al. Sequence-dependent Denaturation Energetics: A Major Determinant in Amyloid Disease Diversity. Proc Natl Acad Sci USA. 2002;99(Suppl 4):16427-32. 7. Hornstrup LS, et al. Genetic Stabilization of Transthyretin, Cerebrovascular Disease, and Life Expectancy. Arterioscler Thromb Vasc Biol. 2013;33(6):1441-7. 8. Liz MA, et al. A Narrative Review of the Role of Transthyretin in Health and Disease. Neurol Ther. 2020;9(2):395-402. 9. Vieira M and Saraiva MJ. Transthyretin: A Multifaceted Protein. Biomol Concepts. 2014;5(1):45-54. 10. Buxbaum JN, Ruberg FL. Transthyretin V122I (pV142I)* Cardiac Amyloidosis: An Age-Dependent Autosomal Dominant Cardiomyopathy Too Common to be Overlooked as a Cause of Significant Heart Disease in Elderly African Americans. Genet Med. 2017;19(7):733-742.
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