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392
Abstracts
mental organizations. Lessons learned from biobanking
efforts, all born of advocacy, for different diseases, using
different models, will be discussed.
FO-4-4
MeCP2 expression in human neocortex
W.E. Kaufman, M.H. Jarrar, S.M. MacDonald
Departments of Pathology, Neurology, Pediatrics, Psychiatry,
and Radiology, The Johns Hopkins University School of
Medicine, and the Kennedy Krieger Institute, Baltimore,
MD, USA
MeCP2 is a member of the methyl-CpG-binding family
of proteins, which is involved in transcriptional repression.
Rett syndrome is a disorder characterized by mental retardation
and autistic features, which is associated in a majority
of cases with mutations within the coding region of the
MeCP2 gene. Considering that MeCP2 mutations are
mainly associated with neurologic disorders, we examined
the pattern of expression of MeCP2 in normal human frontal
neocortex by immunoblotting using a battery of antibodies
targeting different domains of the protein. In
addition to our recent demonstration of extra-nuclear,
mainly postsynaptic, MeCP2 immunoreactivity, we found
that MeCP2 is expressed as two distinctive bands of ,75
and ,100 kd. Both bands are better defined under higher
denaturing conditions, suggesting that MeCP2 has a
complex conformation in brain. The most abundant 75
kd MeCP2 band is the predominant one in nuclear fractions
and in bovine whole cortex, our technical control
mammalian sample, with 75:100 kd ratios of 2.59 and
2.23, respectively. In contrast, in human whole homogenates
the 100 kd is expressed in a higher proportion with
respect to the 75 kd (75:100 kd ratio: 1.80). This suggests
that the 100 kd MeCP2, at this point of uncertain origin
(posttranslational modification versus different isoform), is
a major component of MeCP2’s extra-nuclear pool. These
findings further support the concept that MeCP2 may link
synaptic activity and other signaling processes with transcriptional
regulation in neurons, a base for the predominant
neurologic involvement shown by individuals with
MeCP2 mutations.
FO-5
Cerebral Palsy (2)
FO-5-1
The normal tissue distribution of the neuroglobin gene
and its expression under hypoxic-ischemic brain damage
H.-Y. Wang, M.-Y. Deng, C.-G. Zhang
Department of Pediatrics the General Hospital of the PLA,
Beijing, China
The globins are proteins famous for their oxygen-carrying
capacity. Two types are known in vertebrates: hemoglobin
and myoglobin. Recently neuroglobin (NGB) has
been identified, mainly in the brain, which is a monomer
with a high oxygen affinity. The globin has a distinct function
similar to myoglobin. To investigate its function to a
greater degree, we initially studied the normal distribution
of the NGB gene in the brain. Using the nucleic acid in situ
hybridization (ISH) and immunohistochemistry method,
we found that NGB mRNA and NGB protein were abundant
in the brain of the normal adult rat. A large number of
NGB mRNA and NGB-immunoreactive (NGB-IR) positive
cells were distributed in the neurons of the rat brain. They
were both distributed in the cytoplasm of neurons. Notably,
the number of NGB mRNA and NGB-IR positive cells in
the cerebral cortex was much higher than in the hippocampus
and cerebellum, especially in the piriform cortex.
Secondly, the dynamic change of expression of NGB
mRNA in cerebral tissue was studied by using an adult
rat ischemic brain damage model and reverse transcription
(RT)-PCR technique. We discovered that the expression of
NGB gene showed a time-dependent pattern. It increased
rapidly at 1 min after ischemia, kept at a high level at 5
min, then returned to normal at 15 min. It then increased
again, but more slowly. Finally, we studied the expression
of NGB mRNA in 13 different tissues of humans. The
result showed that there was high expression in fetal
kidneys and normal adult livers, apart from the brain.
FO-5-2
Types and complications of 1192 cases with cerebral
palsy
Y.-K. Li a ,Q.Li b , Y.-J. Liu b , X.-S. Wen a
a Qingdao Children Hospital, Qingdao City, China; b Rehabilitation
Center for Cerebral Palsy, Jiamusi University,
China
We analyzed types and complications of 1192 cases with
CP, come around China and hospitalized in Rehabilitation
Center for Cerebral palsy, Jiamusi University during 1986–
1997. Ages of those patients varied from 5 months to 7
years 3 months, averaged 3 years 2 months. Purpose: This
report provides basic data for studies of cerebral palsy in
China. Results: On types of CP, spastic was 715 cases
(60.0%), athetosis 287 (24.1%), mixed types 113 (9.5%),
atonic 58 (4.9%), ataxia nine (0.7%), unclassified ten
(0.8%), similar to the investigation of Narabayashi. In the
spastic CP, quadriplegia was most, 375 cases (52.5%),
diplegia 135 (18.9%), hemiplegia 83 (11.6%), double
hemiplegia 77 (10.8%), triplegia 21 (7.3%), paraplegia
13 (1.8%) and monoplegia 11 (1.5%). In the athetosis,
quadriplegia was predominant (284 cases, 99.0%), only
three cases of hemiplegia (1.0%). Complications of CP
chiefly were MR, 567 cases (47.6%), principally found in
spastic quadriplegia (53.6%), triplegia (57.1%), double
hemiplegia (46.8%), athetosis (48.1%) and mixed type