Th1L04 Misfolded protein disorders


Medicine Y1 (Theme 1 | Cells and organelles) Flashcards on Th1L04 Misfolded protein disorders, created by Emma Allde on 16/08/2016.
Emma Allde
Flashcards by Emma Allde, updated more than 1 year ago
Emma Allde
Created by Emma Allde over 7 years ago

Resource summary

Question Answer
Sickle cell disease is due to which mutation resulting in abnormally folded haemoglobin (HbS) Glu -> Val
The misfolded HbS results in what characteristic Polymerisation
Polymerised haemoglobin have what characteristics Crystallised chain structures which disrupt the membrane of red blood cells
Alzheimer's disease is mostly likely the result of what formation of stable aggregations of proteins and amyloid proteins forming plaques
Creutzfeldt-Jakob's disease is the result of what stable aggregations of proteins called prions
What is a prion A protein that can fold in multiple, structurally distinct ways, at least one of which is transmissible to other prion proteins
Where are proteins found on the surface of cells
Prion protein polymerisation results in conversion of normal PrPC into what pathogenic form PrSC
Polymerisation of the pathogenic form (PrPSC) in CJD forms what fibrils (aggregates)
Structure of normal prion proteins (PrPC) Alpha helical, susceptible to proteolysis
Structure of pathogenic prion proteins (PrPSC) Beta sheet becomes present, protease resistant so it accumulates Identical amino-acid sequence to normal prion protein
Neurological symptoms of CJD (5) Difficulties with walking, slurred speech, numbness, dizziness, visual problems
Psychological symptoms of CJD (5) Severe depression, withdrawal, anxiety, irritability, insomnia
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