Familial amyloid polyneuropathy

Familial amyloid polyneuropathy
Other namesFAP, hereditary transthyretin amyloidosis (hATTR), Corino de Andrade's disease
Familial amyloid polyneuropathy has an autosomal dominant pattern of inheritance.
SpecialtyNeurology
Diagnostic methodGenetic testing
MedicationTafamidis, patisiran

Familial amyloid polyneuropathy, also called hereditary transthyretin amyloidosis (hATTR),[1][2] or Corino de Andrade's disease,[3] is an autosomal dominant[4] neurodegenerative disease. It is a form of amyloidosis, and was first identified and described by Portuguese neurologist Mário Corino da Costa Andrade, in 1952.[5] FAP is distinct from senile systemic amyloidosis (SSA), which is not inherited, and which was determined to be the primary cause of death for 70% of supercentenarians who have been autopsied.[6] FAP can be ameliorated by liver transplantation.

  1. ^ Ruberg, Frederick L.; Grogan, Martha; Hanna, Mazen; Kelly, Jeffery W.; Maurer, Mathew S. (11 June 2019). "Transthyretin Amyloid Cardiomyopathy: JACC State-of-the-Art Review". Journal of the American College of Cardiology. 73 (22): 2872–2891. doi:10.1016/j.jacc.2019.04.003. PMC 6724183.
  2. ^ Kaku, Michelle; Berk, John L. (October 2019). "Neuropathy Associated with Systemic Amyloidosis". Seminars in Neurology. 39 (05): 578–588. doi:10.1055/s-0039-1688994.
  3. ^ Online Mendelian Inheritance in Man (OMIM): Amyloidosis, hereditary, transthyretin-related - 105210
  4. ^ Ando Y, Ueda M (May 2008). "Novel methods for detecting amyloidogenic proteins in transthyretin related amyloidosis". Frontiers in Bioscience. 13 (13): 5548–58. doi:10.2741/3098. PMID 18508604.
  5. ^ Andrade C (September 1952). "A peculiar form of peripheral neuropathy; familiar atypical generalized amyloidosis with special involvement of the peripheral nerves". Brain. 75 (3): 408–27. doi:10.1093/brain/75.3.408. PMID 12978172.
  6. ^ Coles LS, Young RD (May 2012). "Supercentenarians and transthyretin amyloidosis: the next frontier of human life extension". Preventive Medicine. 54 Suppl (Suppl): S9–11. doi:10.1016/j.ypmed.2012.03.003. PMID 22579241.