Topic:

Protein Mis-folding
(Amylodoisis)
Presented by:
Khadija khan(75)
 PROTEIN MISFOLDING

• Protein Folding Complex process

• Sometimes lead to Improper folding of molecules
• Cell has two line of Defense against such molecules.
•
Chaperons
Protease

• Control system is not

perfect which leads to diseases
 AMYLOID DISEASES/Amylodoisis

“Amyloids refers to the abnormal fibrous, extracellular,

proteinaceous deposits found in organs and
tissues. Amyloid is insoluble & consisting of β-pleated
sheets”
Causes:
o Mutation in particular gene
o Abnormal Proteolytic cleavege of normal proteins
Diseases:
Parkinson Alzheimer Huntington
 Alzheimer's disease

“Age-related neurodegenerative disorder”

CAUSE:
“Abnormal build-up of proteins in
and around brain cells”
o One of the proteins involved is
called amyloid, deposits of which
form plaques around brain cells
(AMYLOID PLAQUES)
 Amyloid plaque
• Dominanant component of plaque that leads to Alzhemier is amyloid β

Extracellular
Amyloid β peptide

40-42 amino acids

• This peptide in residues

β-pleated configuration is neuro-toxic.

• X-ray crystallography and infrared
spectroscopy demonstration.
Amyloid plague formation
 Abnormal Tau accumulation
• Tau is a Structural protein found inside nerve cell
• Beside Amyloid ,accumulation of neuro-fibrillary tangles of
tau inside neurons block normal functions and lead to
Alzheimer.