Elevation of CRABP-I in the cerebrospinal fluid of patients with Moyamoya disease.
OBJECTIVE
The etiology of moyamoya disease (MMD) remains obscure. This study was undertaken to identify specific proteins associated with the pathogenesis of MMD.


METHODS
We studied cerebrospinal fluid (CSF) from 20 patients with angiographically confirmed MMD (4 boys and 16 girls; age range, 3 to 13 years; mean, 7.5 years) and 4 control patients with cerebral palsy who underwent selective dorsal rhizotomy (2 boys and 2 girls; age range, 5 to 10 years; mean, 7.3 years). CSF proteins were analyzed by 2-dimensional polyacrylamide gel electrophoresis, and protein identification was performed by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry. The presence of specific CSF protein in patients with MMD was confirmed by Western blotting. In addition, cerebral CSF was also tested in 7 patients who had other brain diseases but no MMD (2 boys and 5 girls; age range, 1 to 12 years; mean, 6.9 years).


RESULTS
We identified 1 polypeptide spot (Mr of 13 to 15 kDa and isoelectric point of 5 to 5.5) that was differentially expressed in the CSF samples of MMD patients (mean optical density intensity, 0.36+/-0.24; range, 0.05 to 0.92) and control spinal CSF samples (mean, 0.03+/-0.04; range, 0 to 0.08; P=0.002). This polypeptide was identified as cellular retinoic acid-binding protein (CRABP)-I. High levels of expression of CRABP-I in the CSF from 17 MMD children were confirmed by Western blotting.


CONCLUSIONS
The analysis of the CSF of MMD patients reveals high CRABP-I expression. The present study suggests that the elevation of CRABP-I in CSF may be a candidate for pathogenesis of MMD.
Kim
Seung-Ki
SK
Division of Pediatric Neurosurgery and Laboratory for Fetal Medicine Research in Clinical Research Institute, Seoul National University Hospital, and Neuroscience Research Institute, Seoul National University Medical Research Center, Korea.
Yoo
Jong-Il
JI
Cho
Byung-Kyu
BK
Hong
Soo Jin
SJ
Kim
Yong-Kook
YK
Moon
Jung-Ae
JA
Kim
Ji Ha
JH
Chung
You-Nam
YN
Wang
Kyu-Chang
KC
D015415:Biomarkers / Q000032:analysis / Q000134:cerebrospinal fluid; D015153:Blotting, Western; D002555:Cerebrospinal Fluid / Q000737:chemistry*; D002556:Cerebrospinal Fluid Proteins / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D015180:Electrophoresis, Gel, Two-Dimensional; D005260:Female; D006801:Humans; D008297:Male; D009072:Moyamoya Disease / Q000134:cerebrospinal fluid* / Q000209:etiology; D018168:Receptors, Retinoic Acid / Q000032:analysis*; D019032:Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization
D015415:Biomarkers; D002556:Cerebrospinal Fluid Proteins; D018168:Receptors, Retinoic Acid; C082927:retinoic acid binding protein I, cellular