MECHANISM OF DISEASE:

Pathogenic TTR is at the source of clinical symptoms and ATTR progression1-8

Accumulation of pathogenic TTR deposits leads to ATTR progression.
TTR SYNTHESIS1, 5-8
ATTR is caused by changes in TTR, a tetrameric protein primarily synthesized in the liver.
TETRAMER DISSOCIATION1, 5-8
TTR tetramers become less stable and dissociate into monomers and fragments.
MONOMER AGGREGATION1, 5-8
TTR monomers and fragments misfold and aggregate into pathogenic amyloid fibrils.
AMYLOID DEPOSITION1, 5-8
TTR amyloid deposits accumulate in the heart, nerves, gastrointestinal tract, and other tissues, causing damage that leads to clinical symptoms.
DISEASE PROGRESSION1, 5-8
Continued accumulation of amyloid deposits results in worsening clinical symptoms over time.
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Learn more about the mechanism of disease behind ATTR

ATTR=transthyretin-mediated amyloidosis; TTR=transthyretin.
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Identifying ATTR Arrow right Arrow right

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References:

  1. Shin SC et al. Mt Sinai J Med. 2012;79(6):733-748.
  2. Koike H et al. Biomedicines. 2019;7(1):11.
  3. Adams D et al. Neurology. 2015;85(8):675-682.
  4. Adams D et al. Curr Opin Neurol. 2016;29(suppl 1):S14-S26.
  5. Sekijima Y. J Neurol Neurosurg Psychiatry. 2015;86(9):1036-1043.
  6. Nativi-Nicolau JN et al. Heart Fail Rev. 2022;27(3):785-793.
  7. Kittleson MM et al. J Am Coll Cardiol. 2023;81(11):1076-1126.
  8. Hazenberg BPC. Rheum Dis Clin North Am. 2013;39(2):323-345.