[Molecular basis of familial and sporadic forms of human prion diseases].

doi:10.1016/s1246-7820(06)80016-9

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[Molecular basis of familial and sporadic forms of human prion diseases].

Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguine - 1 Jan 1994

Laplanche J L

Abstract excerpt

Prion diseases, also known as transmissible subacute spongiform encephalopathies (TSSE), are rare neurodegenerative disorders of both humans and animals. Their biochemical hallmark is an accumulation in the brain of an abnormal form of the host-encoded prion protein (PrP). This pathological accumulation could result from a protein conformational change under the influence of unknown factors. The normal function...

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[Molecular basis of familial and sporadic forms of human prion diseases].

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