BSC 310 Lecture Notes - Lecture 37: Pyrimidine, Silent Mutation, Transposable Element

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Host cells contain a gene (prnp) which encodes the native form of the prion protein in healthy individuals (cid:1) Pathogenic form of the protein is prpsc (sc for scrapie, the first prion disease discovered) (cid:1) Prpsc is identical in amino acid sequence to prpc but has a different conformation (the native is largely alpha helices while the pathogenic forms contain more beta-pleated sheets (cid:1) Native prion proteins are converted to the pathogenic form by inducing misfolding when a prpsc form enters a host cell (cid:1) As pathogenic prion proteins accumulate, they form insoluble aggregates in nerve cells (cid:1) Leads to disease symptoms that are neurological and due to the destruction of brain or related nervous tissue (cid:1) Prpc functions as a cytoplasmic membrane glycoprotein but is not required for survival. Membrane attachment of prpsc is necessary for disease symptoms. Exposure to prions can be infectious, sporadic (random misfolding in (cid:1) an individual), or inherited (cid:1)

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