NR ASIG
AU Al Bersaoui,R.; Robert,I.; Lutz,Y.; Blanc,F.; Sommermeyer-Leroux,G.; Shibaguchi,H.; Aunis,D.; Fuchs,J.P.
TI Purkinje-cell degeneration in prion protein-deficient mice is associated with a cerebellum-specific Doppel protein species signature
QU FEBS Letters 2005 May 9; 579(12): 2715-21
IA http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6T36-4G05H5B-3&_user=10&_coverDate=05%2F09%2F2005&_rdoc=29&_fmt=full&_orig=browse&_srch=doc-info(%23toc%234938%232005%23994209987%23594361%23FLA%23display%23Volume)&_cdi=4938&_sort=d&_docanchor=&_ct=36&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=33267c809179d976872792ffca2b5c5b
PT journal article
AB PrPc (cellular prion protein) and Doppel are antagonizing proteins, respectively neuroprotective and neurotoxic. Evidence for Doppel neurotoxicity came from PrPc-deficient (Prnp(0/0)) mouse lines developing late onset Purkinje-cell degeneration caused by Doppel overexpression in brain. To address the molecular underpinnings of this cell-type specificity, we generated Doppel N-terminal-specific antibodies and started to examine the spatio-temporal expression of Doppel protein species in Ngsk Prnp(0/0) brain. Although Doppel overexpression is ubiquitous, Western analyses of normal and deglycosylated protein extracts revealed cerebellar patterns distinct from the rest of the brain, supporting the idea that neurotoxicity might be linked to a particular Doppel species pattern. Furthermore, our newly raised antibodies allowed the first Doppel immunohistochemical analyses in brain, showing a distribution in Prnp(0/0) cerebellum similar to PrPc in wild type.
MH Animals; Blotting, Western; Cerebellum/*chemistry; Immunohistochemistry; Mice; Mice, Knockout; Nerve Degeneration/*pathology; Polymerase Chain Reaction; Prions/genetics/*metabolism; Purkinje Cells/*pathology; Recombinant Proteins/metabolism; Research Support, Non-U.S. Gov't
AD Roméo Al Bersaoui, Isabelle Robert, Yves Lutz, Frédéric Blanc, Ghislaine Sommermeyer-Leroux, Dominique Aunis, Jean-Paul Fuchs (address: fuchs@neurochem.u-strasbg.fr), Unité 575 INSERM, Physiopathologie du Système Nerveux, 5 rue Blaise Pascal, 67084 Strasbourg Cedex, France; Hirotomo Shibaguchi, Fukuoka University School of Medicine, Department of Biochemistry, 7-45-1 Nanakuma Jonan-ku, Fukuoka 814-0180, Japan
SP englisch
PO Niederlande