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Amyloidosis- introduction

Amyloidosis- introduction


AMYLOIDOSIS

  • Amyloidosis is a group of diseases characterised by extracellular deposition of fibrillar insoluble proteinaceous substance called amyloid.
  • The term amyloid was coined by Lister.
  • Amyloidosis is basically a disorder of protein misfolding.
  • Etiology of amyloidosis is unknown.

Physical & Chemical properties of Amyloid-

  • Amyloid is composed of 2 main types of complex proteins-
  1. Fibril protein- 95% of amyloid
  2. Non- fibrilar component- P component & 5% amyloid.
  • Amyloid is formed of 4-6 fibrils of 7.5- 10 nm diameter.
  • Dye congo redbinds to fibrils & forms red- green dichroism on amyloid deposits.
  • By X- ray crystallography & infra red spectroscopy – cross β pleated sheet produce 1000 A0 periodicity.  

Exam Important

  • Amyloidosis is a group of diseases characterised by extracellular deposition of fibrillar insoluble proteinaceous substance called amyloid.
  • The term amyloid was coined by Lister.
  • Amyloidosis is basically a disorder of protein misfolding.
  • Etiology of amyloidosis is unknown.

Physical & Chemical properties of Amyloid-

  • Amyloid is composed of 2 main types of complex proteins-
  1. Fibril protein- 95% of amyloid
  2. Non- fibrilar component- P component & 5% amyloid.
  • Amyloid is formed of 4-6 fibrils of 7.5- 10 nm diameter.
  • Dye congo red binds to fibrils & forms v
  • By X- ray crystallography & infra red spectroscopy – cross β pleated sheet produce 1000 A0 periodicity.

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