PMID- 8764595
OWN - NLM
STAT- MEDLINE
DCOM- 19960919
LR  - 20220321
IS  - 0022-3042 (Print)
IS  - 0022-3042 (Linking)
VI  - 67
IP  - 2
DP  - 1996 Aug
TI  - Apoptosis as the mechanism of neurodegeneration in Batten's disease.
PG  - 677-83
AB  - Batten's disease is a genetic neurodegenerative disease of childhood. Its 
      hallmarks are retinitis pigmentosa and neuronal degeneration. As some types of 
      photoreceptor death in mice are mediated by apoptosis, we investigated whether 
      apoptosis is responsible for retinal and neuronal degeneration in the late 
      infantile and juvenile forms of Batten's disease. Using the terminal dUDP nick 
      end-labeling (TUNEL) staining method, we detected apoptotic neuronal cells in 
      brain from patients and a canine model and in brain and retina from an ovine 
      model for Batten's disease. We confirmed apoptosis by flow cytometry, electron 
      microscopy, and DNA laddering. This is the first inherited neurodegenerative 
      disease involving brain and retina in which apoptosis has been established as the 
      mechanism of neuronal and photoreceptor cell death in both humans and animal 
      models.
FAU - Lane, S C
AU  - Lane SC
AD  - Department of Pediatrics, Duke University Medical Center, Durham, North Carolina, 
      USA.
FAU - Jolly, R D
AU  - Jolly RD
FAU - Schmechel, D E
AU  - Schmechel DE
FAU - Alroy, J
AU  - Alroy J
FAU - Boustany, R M
AU  - Boustany RM
LA  - eng
GR  - R01 NS30170-04/NS/NINDS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - England
TA  - J Neurochem
JT  - Journal of neurochemistry
JID - 2985190R
SB  - IM
MH  - Animals
MH  - *Apoptosis
MH  - Autopsy
MH  - Cerebral Cortex/pathology
MH  - Disease Models, Animal
MH  - Dogs
MH  - Humans
MH  - *Nerve Degeneration
MH  - Neuronal Ceroid-Lipofuscinoses/*pathology
MH  - Retina/pathology
MH  - Sheep
EDAT- 1996/08/01 00:00
MHDA- 1996/08/01 00:01
CRDT- 1996/08/01 00:00
PHST- 1996/08/01 00:00 [pubmed]
PHST- 1996/08/01 00:01 [medline]
PHST- 1996/08/01 00:00 [entrez]
AID - 10.1046/j.1471-4159.1996.67020677.x [doi]
PST - ppublish
SO  - J Neurochem. 1996 Aug;67(2):677-83. doi: 10.1046/j.1471-4159.1996.67020677.x.