Prion‐like protein Doppel expression is not modified in scrapie‐infected cells and in the brains of patients with Creutzfeldt–Jakob disease

FEBS Letters

Laplanche, J.-L³  Sazdovitch, V⁴  Beaudry, P³  Creminon, C²  Lehmann, S¹  Sorgato, M.C⁵  Volland, H²  De Gassart, A¹  Peoc'h, K³ 

[1] Biologie des Encéphalopathies Spongiformes Transmissibles IGH du CNRS, UPR 1142, 141 rue de la Cardonille, 34396 Montpellier Cedex 5, France;Service de Pharmacologie et d'Immunologie, CEA Saclay, 91191 Gif Sur Yvette Cedex, France;Service de Biochimie et Biologie Moléculaire, Hôpital Lariboisière, 2 Rue A. Paré, 75475 Paris, France;Laboratoire de Neuropathologie R. Escourolle, Groupe hospitalier Pitié-Salpêtrière, INSERM U 360, 47 bd de L'Hôpital, 75013 Paris, France;Dipartimento di Chimica Biologica, Universita di Padova, 35121 Padova, Italy

关键词: Prion;    Doppel;    Creutzfeldt–Jakob disease;    Brain;    mRNA;    Immunoassay;    CJD;    Creutzfeldt–Jakob disease;    Dpl;    Doppel protein;    mAb;    monoclonal antibody;    OD;    optical density;    pAb;    polyclonal antibody;    PK;    proteinase K;    PrPc;    cellular prion protein;    PrPSc;    scrapie prion protein;   

DOI  :  10.1016/S0014-5793(03)00012-7

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

Doppel protein has been discovered in prnp knock-out mouse lines, with overproduction of this protein in the brain causing ataxia and neurodegeneration. We investigated whether Doppel expression (i) affected or was affected by the course of prion propagation in neuroblastoma cells, or (ii) modulated Creutzfeldt–Jakob disease pathogenesis. No change in Doppel production was detected in N2a cells, before or after infection. Transient murine Doppel gene expression had no effect on N2a viability or PrP^Sc production. A sensitive immunometric assay revealed low levels of Doppel in human brain, reflecting weak transcription of the corresponding gene. No difference in brain Doppel levels was observed between Creutzfeldt–Jakob disease patients and controls, adding further evidence that Doppel is unlikely to be involved in prion disease pathogenesis.

【 授权许可】

Unknown