PMID- 10802792
OWN - NLM
STAT- MEDLINE
DCOM- 20000530
LR  - 20190514
IS  - 0028-3878 (Print)
IS  - 0028-3878 (Linking)
VI  - 54
IP  - 9
DP  - 2000 May 9
TI  - CSF insulin-like growth factor-1 in infantile neuronal ceroid lipofuscinosis.
PG  - 1828-32
AB  - BACKGROUND: Infantile neuronal ceroid lipofuscinosis (INCL) is a progressive 
      encephalopathy in which the patients are severely disabled by the age of 3 years. 
      It is characterized by cerebral atrophy, selective loss of cortical neurons, and 
      secondary loss of axons and myelin sheaths of the white matter. INCL has been 
      shown to result from a palmitoyl protein thioesterase deficiency. The authors 
      suggested that insulin-like growth hormones and apoptosis might play a role in 
      the pathogenesis of INCL. METHODS: The authors measured insulin-like growth 
      factor-1 (IGF-1) and IGF binding protein 3 (IGFBP-3) in the CSF of patients with 
      INCL by radioimmunoassay at an early stage when myelin was starting to diminish. 
      RESULTS: The authors found low CSF IGF-1 but normal IGFBP-3 in patients with INCL 
      compared with control subjects. Also, they observed apoptotic cell death in 
      biopsies of INCL patients. CONCLUSIONS: Because the IGF system seems to be 
      important for early brain development, myelination, and neuroprotection, the 
      authors suggest that the pathology in INCL may be associated with low CSF IGF-1.
FAU - Riikonen, R
AU  - Riikonen R
AD  - University Hospital of Kuopio, Helsinki, Finland. raili.riikonen@kolumbus.fi
FAU - Vanhanen, S L
AU  - Vanhanen SL
FAU - Tyynela, J
AU  - Tyynela J
FAU - Santavuori, P
AU  - Santavuori P
FAU - Turpeinen, U
AU  - Turpeinen U
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Neurology
JT  - Neurology
JID - 0401060
RN  - 0 (Insulin-Like Growth Factor Binding Protein 3)
RN  - 67763-96-6 (Insulin-Like Growth Factor I)
SB  - IM
MH  - Apoptosis/physiology
MH  - Biopsy
MH  - Child, Preschool
MH  - Female
MH  - Humans
MH  - Infant
MH  - Insulin-Like Growth Factor Binding Protein 3/cerebrospinal fluid
MH  - Insulin-Like Growth Factor I/*cerebrospinal fluid
MH  - Male
MH  - Myelin Sheath/pathology
MH  - Neuronal Ceroid-Lipofuscinoses/cerebrospinal fluid/*diagnosis/pathology
MH  - Neurons/pathology
MH  - Radioimmunoassay
EDAT- 2000/05/10 09:00
MHDA- 2000/06/03 09:00
CRDT- 2000/05/10 09:00
PHST- 2000/05/10 09:00 [pubmed]
PHST- 2000/06/03 09:00 [medline]
PHST- 2000/05/10 09:00 [entrez]
AID - 10.1212/wnl.54.9.1828 [doi]
PST - ppublish
SO  - Neurology. 2000 May 9;54(9):1828-32. doi: 10.1212/wnl.54.9.1828.