Download - Protein Folding Diseases
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Protein folding diseases
L.Revathi
14MG12
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What is protein folding?
• Proteins are the basis of how biology gets
things done.
• In order to carry out their function (e.g. as
enzymes or antibodies), they must take on a
particular shape Protein folding.
• ! small error in the folding process results in a
misfolded structure which results in many
diseases.
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What happens if proteins don"t fold
correctly?
(!) #ati$e polypeptide chain composed of mainly %&helical secondarystructure.
(') isfolding causes con$ersion of %&helical structure to &pleated sheets and
(*) final misfolded structure of polypeptide chain contains mostly &pleated
sheets.
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*ellular conse+uences of protein aggregation
• Loss-of-function pathogenesis if misfolded
proteins are prematurely degraded by P-* system
protein deficiency disease
• Gain-of-function pathogenesis if misfolded
proteins are not eliminated but accumulated instead
disease pathology
toicity
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Protein dimerization / oligomerization.
• isfolded monomers forming aggregate
through intermolecular hydrogen bonding
interaction leading to &sheet formation.
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!lzheimer"s disease
• 0he etracellular deposition of beta amyloid
pla+ues and neurofibrillary tangles (#10) in
the brain.
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Prion diseases
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0ransmissable spongiform encephalopathies
(023s)
• *reutzfeldt4akob disease
• 'o$ine spongiform
encephalopathy (mad cow disease)
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Protein misfolding diseases
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1ate of misfolded proteins
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0he role of chaperones in rescuing protein
conformational defects
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• Mutational efect in human proteins
are corrected by molecular, chemicaland pharmacological chaperones.
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0hank you