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Physical nature of amyloid


1. On election microscope 🔬


2. On X-ray crystallography and infrared spectroscopy


3.


1. On EM - amyloid is seen to be made up largely of continuous, non branching fibrils with a diameter of ~7.5 - 10 nm


This EM structure is identical in all types of amyloidosis


2. On X-ray crystallography and infrared spectroscopy show a characteristic cross-β-pleated sheet conformation



Amyloid material consist of ?

Fibril proteins 95%


remaining 5% p- component and other glycoproteins



The major forms of amyloid


List some minor forms

1. AL amyloid


2. AA amyloid


3. Aβ amyloid



Minor


1. TTR - transthyretin - normal serum protein that binds and transports thyroxine and retinol


2. β2 - microglobulin - a component of MHC class I Molecules and a normal serum protein, has been identified as the amyloid Fibril subunit Aβ2m in amyloidosis that complicates the course of patients on long term haemodialysis


3. Prion proteins - CNS, local amyloidosis

About AL protein


1. Is made up of?

1. Complete immunoglobulin light chains, the amino-terminal fragments of light chains or both


2. Most are lamda chains or their fragments


But in some cases kappa k - chains have been identified


3. The amyloid Fibril protein of the AL type is produced from Free If light chains secreted by a monoclonal population of plasma cells and it's deposition is associated with certain forms of plasma cells tumors

About AA amyloid Fibril

1. MW - 8500 ,


2. 76 amino acid residues


Serum amyloid o may contribute to amyloid deposition by?

Stabilizing the fibrils


and decreasing their clearance