PMID- 11001810
OWN - NLM
STAT- MEDLINE
DCOM- 20001113
LR  - 20211203
IS  - 1096-7192 (Print)
IS  - 1096-7192 (Linking)
VI  - 71
IP  - 1-2
DP  - 2000 Sep-Oct
TI  - Developmental changes in the expression of neuronal ceroid lipofuscinoses-linked 
      proteins.
PG  - 190-4
AB  - Neuronal ceroid lipofuscinoses (NCL) form a distinct group of storage diseases 
      where the normal development of the central nervous system is interrupted and 
      neurons of the neocortex begin to degenerate. Mutations in genes encoding three 
      lysosomal enzymes are the causes for three early-onset forms of NCLs: 
      palmitoyl-protein thioesterase 1 (PPT1) is deficient in human infantile NCL, 
      tripeptidyl peptidase 1 (TTP1) in late-infantile NCL, and cathepsin D in 
      congenital ovine NCL. We wanted to compare the developmental expression profiles 
      of these enzymes in rat brain. In conclusion, the PPT1 expression pattern 
      differed from the two other lysosomal enzymes implicated in NCL diseases, thus 
      suggesting a distinctive role for PPT1 in brain development.
CI  - Copyright 2000 Academic Press.
FAU - Suopanki, J
AU  - Suopanki J
AD  - Institute of Biomedicine, University of Helsinki, Finland. 
      jaana.suopanki@helsinki.fi
FAU - Partanen, S
AU  - Partanen S
FAU - Ezaki, J
AU  - Ezaki J
FAU - Baumann, M
AU  - Baumann M
FAU - Kominami, E
AU  - Kominami E
FAU - Tyynela, J
AU  - Tyynela J
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - United States
TA  - Mol Genet Metab
JT  - Molecular genetics and metabolism
JID - 9805456
RN  - 0 (DNA Primers)
RN  - 0 (RNA, Messenger)
RN  - 0 (Tripeptidyl-Peptidase 1)
RN  - EC 3.1.2.- (Thiolester Hydrolases)
RN  - EC 3.1.2.22 (palmitoyl-protein thioesterase)
RN  - EC 3.4.- (Endopeptidases)
RN  - EC 3.4.- (Serine Proteases)
RN  - EC 3.4.11.- (Aminopeptidases)
RN  - EC 3.4.14.- (Dipeptidyl-Peptidases and Tripeptidyl-Peptidases)
RN  - EC 3.4.23.5 (Cathepsin D)
SB  - IM
MH  - Aminopeptidases
MH  - Animals
MH  - Base Sequence
MH  - Cathepsin D/genetics/metabolism
MH  - Central Nervous System/enzymology/growth & development/pathology
MH  - DNA Primers/genetics
MH  - Dipeptidyl-Peptidases and Tripeptidyl-Peptidases
MH  - Endopeptidases/genetics/metabolism
MH  - Gene Expression Regulation, Developmental
MH  - Humans
MH  - Lysosomes/enzymology
MH  - Mutation
MH  - Nerve Degeneration
MH  - Neuronal Ceroid-Lipofuscinoses/*enzymology/genetics/pathology
MH  - RNA, Messenger/genetics/metabolism
MH  - Rats
MH  - Serine Proteases
MH  - Thiolester Hydrolases/genetics/metabolism
MH  - Tripeptidyl-Peptidase 1
RF  - 36
EDAT- 2000/09/26 11:00
MHDA- 2001/02/28 10:01
CRDT- 2000/09/26 11:00
PHST- 2000/09/26 11:00 [pubmed]
PHST- 2001/02/28 10:01 [medline]
PHST- 2000/09/26 11:00 [entrez]
AID - S1096-7192(00)93071-3 [pii]
AID - 10.1006/mgme.2000.3071 [doi]
PST - ppublish
SO  - Mol Genet Metab. 2000 Sep-Oct;71(1-2):190-4. doi: 10.1006/mgme.2000.3071.