FUNPEP: Information system for "low complexity sequence regions".

Copyright (C) 1999, FUNPEP.

Introduction.

This part of the FUNPEP project is a bit different from all the others. The peptides on these pages were not chosen because of some kind of sequence similarity, what is more, they hardly have any. Their common, and very starnge property is the ability to form amyloid plaques (or fibrils). The exact structure and the formation of these supermolacular structures are still subject of research, but there are lots of promising results.
As a part of the FUNPEP project, we made a small collection of peptides, which are known to form these amyloid plaques. Sequences, including respective animal analogues, were extracted from SWISSPROT, and aligned. These sequences and some words about the peptides can be found under the links in the table below. Some molecular modelling was also perfomed, to show some possible structures of amyloids. The result of modelling is here.

The medical points of amyloidosis was not in our interest, if you want to find information about that, go here.

What is amyloid formation?

Amyloid plaque (or fibril) formation refers to an in vivo process in which one of the human amyloidogenic proteins abnormally self assembles into a fibril 60-100 angstrom in width and of a variable length; the fibril has a characteristic cross-beta repeat structure where the individual beta strands composing the fibril are oriented perpendicular to the long axis of the fibril. The amyloidogenic proteins exhibit little sequence or structural homology, yet they are able to make amyloid fibrils of similar structure, as discerned from fiber X-ray diffraction patterns, their morphology in electron micrographs, and their ability to bind certain dyes (e.g. Congo Red) and exhibit birefringence. More information about the structure of amyloid fibers can be found here, about the dye-binding property here.

Known amyloidogenic peptides

Amyloidogenic diseases and proteins are reviewed by J.W. Kelly in Curr. Opin. Struct. Biol. 6, 11-17. (1996):

CJD  spongiform encepalopathies  prion protein fragments 
APP  Alzheimer  beta protein fragment 1-40/43
HRA  hemodialysis-related amyloidosis  beta-2 microglobin*
PSA  primary systmatic amyloidosis  immunoglobulin light chain and fragments
SAA 1  secondary systmatic amyloidosis  serum amyloid A 78 residue fragment
FAP I**  familial amyloid polyneuropathy I  transthyretin fragments, 50+ allels
FAP III  familial amyloid polyneuropathy III  apolipoprotein A-1 fragments
CAA  cerebral amyloid angiopathy  cystatin C minus 10 residues
FHSA  Finnish hereditary systemic amyloidosis  gelsolin 71 aa fragment
IAPP  type II diabetes  islet amyloid polypeptide fragment (amylin)
ILA  injection-localized amyloidosis  insulin
CAL  medullary thyroid carcinoma  calcitonin fragments
ANF  atrial amyloidosis  atrial natriuretic factor
NNSA  non-neuropathic systemic amylodosis  lysozyme and fragments
HRA  hereditary renal amyloidosis  fibrinogen fragments
* homologous to immunoglobin, so a predicted paralogous disease.
** also called senile systemic amyloidosis, prealbumin is synonymous with transthyretin.
The huntingtin peptide of Huntington's disease also known as an amyloid-forming one. The main differece is that its aggregation is caused by a long repeat of glutamins. Because of this we left it out from our studies.


Gergo Kiss 24th May 1999 EMBL/Szeged