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 * Recognizing ATTR-CM
   * ATTR-CM Diagnosis
   * ATTR-CM Red Flags
   * ATTR-CM Subtypes
   * Sex Differences
 * Managing ATTR‑CM
   * Managing ATTR-CM
   * Monitoring Progression
   * Quality of Life
   * ATTR-CM Biomarkers
 * ATTR-CM Resources
   * Helpful Articles
   * Resources to Share With Patients
   * Glossary

Stay Informed
 * Recognizing ATTR-CM
   * ATTR-CM Diagnosis
   * ATTR-CM Red Flags
   * ATTR-CM Subtypes
   * Sex Differences
 * Managing ATTR‑CM
   * Managing ATTR-CM
   * Monitoring Progression
   * Quality of Life
   * ATTR-CM Biomarkers
 * ATTR-CM Resources
   * Helpful Articles
   * Resources to Share With Patients
   * Glossary


TTR DESTABILIZATION:


THE SOURCE OF ATTR‑CM

Destabilization of the transthyretin (TTR) protein, also known as prealbumin,
leads to low levels of serum TTR and has been correlated with earlier disease
onset and poor clinical outcomes in people with transthyretin amyloid
cardiomyopathy (ATTR‑CM) — a relentlessly progressive disease.*,1-3

*Also known as ATTR cardiomyopathy, or ATTR cardiac amyloidosis (ATTR-CA).

Learn More

This site is intended for US Healthcare Professionals only.


WHAT IS TTR?

TTR, also known as prealbumin, plays a role in processes vital to human
health:4,5

 * Highly conserved transport protein produced in the liver
 * Distributes thyroxine and vitamin A throughout the body
 * Supports memory, neuroprotection, and cognitive function

Click play to learn more about TTR.


TTR DESTABILIZATION IN ATTR‑CM

*Amyloid fibrils can also accumulate in areas of the body
outside of the heart.6,7


TTR DESTABILIZATION IS THE ROOT CAUSE OF ATTR‑CM6,7

Factors such as aging or TTR gene variants can trigger a cascade of structural
changes that destabilize the configuration of circulating TTR tetramers.6 These
changes may cause the tetramers to break apart and misfold.7 Misfolded TTR
proteins can aggregate into toxic amyloid fibrils that cause signs and symptoms
indicative of transthyretin amyloidosis.7,8


TTR STABILITY IMPACTS DISEASE SEVERITY


GREATER TTR DESTABILIZATION IS CORRELATED WITH EARLIER DISEASE ONSET AND
INCREASED DISEASE SEVERITY1

TTR gene variants strongly influence the rate of TTR tetramer dissociation,
which is required for amyloid formation.1 Most known variants destabilize the
tetramer, increasing the amyloidogenic potential of TTR and leading to poor
clinical outcomes.1 However, variants shown to stabilize the tetramer have also
been identified.8 These variants prevent amyloidogenesis by dramatically slowing
the rate of tetramer dissociation and have been associated with disease
protection.1,8

The most beneficial TTR gene variant identified to date is T119M, which has been
shown to confer protection against the development of ATTR‑CM even when
coinherited with variants associated with poor outcomes1

Relative tetramer stability based on denaturation midpoints measured via an in
vitro dissociation/unfolding assay.1

The most beneficial TTR gene variant identified to date is T119M, which has been
shown to confer protection against the development of ATTR‑CM even when
coinherited with variants associated with poor outcomes1

Stay Informed
References:
1.

Hammarström P, et al. Proc Natl Acad Sci U S A. 2002;99 Suppl 4(Suppl
4):16427-32. 2. Hanson JLS, et al. Use of Serum Transthyretin as a Prognostic
Indicator and Predictor of Outcome in Cardiac Amyloid Disease Associated With
Wild-Type Transthyretin. Circ Heart Fail. 2018;11(2):e004000. 3. Lane T, et al.
Natural History, Quality of Life, and Outcome in Cardiac Transthyretin
Amyloidosis. Circulation. 2019;140(1):16-26. 4. Liz MA, et al. A Narrative
Review of the Role of Transthyretin in Health and Disease. Neurol Ther.
2020;9(2):395-402. 5. Vieira M, Saraiva MJ. Transthyretin: A Multifaceted
Protein. Biomol Concepts. 2014;5(1):45-54. 6. Kittleson MM, et al. American
Heart Association Heart Failure and Transplantation Committee of the Council on
Clinical Cardiology. Cardiac Amyloidosis: Evolving Diagnosis and Management: A
Scientific Statement From the American Heart Association. Circulation.
2020;142(1):e7-e22. 7. Ruberg FL, et al. Transthyretin Amyloid Cardiomyopathy:
JACC State-of-the-Art Review. J Am Coll Cardiol. 2019;73(22):2872-2891. 8.
Hornstrup LS, et al. Genetic Stabilization of Transthyretin, Cerebrovascular
Disease, and Life Expectancy. Arterioscler Thromb Vasc Biol. 2013;33(6):1441-7.

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