<prompt> Which type of amyloidosis is caused by mutation of the transthyretin protein?Familial Mediterranean feverFamilial amyloidotic polyneuropathyDialysis associated amyloidosisPrion protein associated amyloidosis  <response>: Familial amyloid polyneuropathy FAP is phenotypically and genetically heterogeneous and is caused by mutations in the genes for transthyretin TTR, apolipoprotein A1, or gelsolin. The majority of patients with FAP have mutations in the TTR gene. Amyloid deposition may be evident in abdominal fat pad, rectal, or nerve biopsies. The clinical features, histopathology, and EDx reveal abnormalities consistent with a generalized or multifocal, predominantly axonal but occasionally demyelinating, sensorimotor polyneuropathy. Must know: Familial Mediterranean fever FMF is the prototype of a group of inherited diseases that are characterized by recurrent episodes of fever with serosal, synol, or cutaneous inflammation and, in some individuals, the eventual development of systemic AA amyloidosis. The innate immune system, with its myeloid effector cells and germline receptors for pathogen-associated molecular patterns and endogenous danger signals, plays a predominant role in the pathogenesis of the autoinflammatory diseases Beta 2-m Amyloidosis is a frequent complication of long-term dialysis hemodialysis or peritoneal dialysis. In fact, Beta 2-m amyloidosis is a major cause of skeletal morbidity in dialysis-dependent patients. Prions are infectious proteins that cause degeneration of the central nervous system CNS. Prion diseases are disorders of protein conformation, the most common of which in humans is called Creutzfeldt-Jakob disease CJD. CJD typically presents with dementia and myoclonus, is relentlessly progressive, and generally causes death within a year of onset. Misfolded prion protein PrPSc is associated with amyloidosis. Ref: Mueller P.S. 2007. Chapter 56. Amyloidosis. In J.B. Imboden, D.B. Hellmann, J.H. Stone Eds, CURRENT Rheumatology Diagnosis Treatment, 2e; Mueller P.S. 2007. Chapter 56. Amyloidosis. In J.B. Imboden, D.B. Hellmann, J.H. Stone Eds, CURRENT Rheumatology Diagnosis Treatment, 2e. 