Abnormality of cerebral gangliosides in Fukuyama type congenital muscular dystrophy.
Compared with DMD cases and non-neuromuscular disease controls, FCMD cases showed a reduction of total gangliosides, and an abnormal, immature ganglioside pattern in the cerebral gray and white matter. However, GM4, which is only found in myelin and oligodendroglia, and is a unique quantitative marker of myelination, was present in a relatively high percentage in the white matter, which showed frontal lobe micropolygyria and diffuse low density on CT and MR T1-imaging.
Izumi
T
T
Department of Pediatrics, Oita Medical University School of Medicine, Japan.
Hara
K
K
Ogawa
T
T
Osawa
M
M
Saito
K
K
Novo
M L
ML
Fukuyama
Y
Y
Takashima
S
S
D000293:Adolescent; D000328:Adult; D015415:Biomarkers; D001921:Brain / Q000473:pathology*; D001923:Brain Chemistry; D002540:Cerebral Cortex / Q000737:chemistry; D002675:Child, Preschool; D005260:Female; D005333:Fetus / Q000473:pathology; D005625:Frontal Lobe / Q000737:chemistry; D005677:G(M1) Ganglioside / Q000378:metabolism; D005732:Gangliosides / Q000378:metabolism*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009136:Muscular Dystrophies / Q000151:congenital / Q000378:metabolism / Q000473:pathology*
D015415:Biomarkers; D005732:Gangliosides; D005677:G(M1) Ganglioside; C028253:ganglioside, GM4