PMID- 10525406
OWN - NLM
STAT- MEDLINE
DCOM- 19991104
LR  - 20101118
IS  - 0022-2836 (Print)
IS  - 0022-2836 (Linking)
VI  - 292
IP  - 4
DP  - 1999 Oct 1
TI  - Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of
      the novel PrP-like protein doppel.
PG  - 797-817
AB  - The novel locus Prnd is 16 kb downstream of the mouse prion protein (PrP) gene
      Prnp and encodes a 179 residue PrP-like protein designated doppel (Dpl). Prnd
      generates major transcripts of 1.7 and 2.7 kb as well as some unusual chimeric
      transcripts generated by intergenic splicing with Prnp. Like PrP, Dpl mRNA is
      expressed during embryogenesis but, in contrast to PrP, it is expressed minimally
      in the CNS. Unexpectedly, Dpl is upregulated in the CNS of two PrP-deficient
      (Prnp(0/0)) lines of mice, both of which develop late-onset ataxia, suggesting
      that Dpl may provoke neurodegeneration. Dpl is the first PrP-like protein to be
      described in mammals, and since Dpl seems to cause neurodegeneration similar to
      PrP, the linked expression of the Prnp and Prnd genes may play a previously
      unrecognized role in the pathogenesis of prion diseases or other illnesses.
FAU - Moore, R C
AU  - Moore RC
AD  - Institute for Neurodegenerative Diseases, Departments of Neurology.
FAU - Lee, I Y
AU  - Lee IY
FAU - Silverman, G L
AU  - Silverman GL
FAU - Harrison, P M
AU  - Harrison PM
FAU - Strome, R
AU  - Strome R
FAU - Heinrich, C
AU  - Heinrich C
FAU - Karunaratne, A
AU  - Karunaratne A
FAU - Pasternak, S H
AU  - Pasternak SH
FAU - Chishti, M A
AU  - Chishti MA
FAU - Liang, Y
AU  - Liang Y
FAU - Mastrangelo, P
AU  - Mastrangelo P
FAU - Wang, K
AU  - Wang K
FAU - Smit, A F
AU  - Smit AF
FAU - Katamine, S
AU  - Katamine S
FAU - Carlson, G A
AU  - Carlson GA
FAU - Cohen, F E
AU  - Cohen FE
FAU - Prusiner, S B
AU  - Prusiner SB
FAU - Melton, D W
AU  - Melton DW
FAU - Tremblay, P
AU  - Tremblay P
FAU - Hood, L E
AU  - Hood LE
FAU - Westaway, D
AU  - Westaway D
LA  - eng
SI  - GENBANK/AF165165
SI  - GENBANK/AF165166
SI  - GENBANK/U29187
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - Netherlands
TA  - J Mol Biol
JT  - Journal of molecular biology
JID - 2985088R
RN  - 0 (GPI-Linked Proteins)
RN  - 0 (Prions)
RN  - 0 (Prnd protein, mouse)
RN  - 0 (RNA, Messenger)
SB  - IM
MH  - Amino Acid Motifs
MH  - Amino Acid Sequence
MH  - Animals
MH  - Ataxia/*genetics
MH  - Base Sequence
MH  - Cell Line
MH  - Central Nervous System/cytology/metabolism/pathology
MH  - Cloning, Molecular
MH  - Embryo, Mammalian/metabolism
MH  - GPI-Linked Proteins
MH  - Gene Deletion
MH  - Glycosylation
MH  - Male
MH  - Mice
MH  - Mice, Inbred BALB C
MH  - Mice, Transgenic
MH  - Molecular Sequence Data
MH  - Prions/chemistry/*genetics/metabolism/physiology
MH  - Purkinje Cells/metabolism/pathology
MH  - RNA, Messenger/analysis/genetics
MH  - Sequence Alignment
MH  - Trans-Splicing/genetics
MH  - Up-Regulation
EDAT- 1999/10/20 00:00
MHDA- 1999/10/20 00:01
CRDT- 1999/10/20 00:00
PHST- 1999/10/20 00:00 [pubmed]
PHST- 1999/10/20 00:01 [medline]
PHST- 1999/10/20 00:00 [entrez]
AID - 10.1006/jmbi.1999.3108 [doi]
AID - S0022-2836(99)93108-X [pii]
PST - ppublish
SO  - J Mol Biol. 1999 Oct 1;292(4):797-817. doi: 10.1006/jmbi.1999.3108.