Comparison of cellular sensitivity to UV killing with neuropsychological impairment in Cockayne syndrome patients.
We studied the neuropsychological function including mental status and motor development, and cellular susceptibility to UV killing in four Cockayne syndrome (CS), of whom three were classic form (type I) and one was congenital form (type II). The results showed that there was no correlation between the age at symptomatic onset of CS neurological disorders and the levels of cellular UV-hypersensitivity and that neuropsychological impairment did not parallel cellular hypersensitivity to UV killing. It was suggested that the cellular UV-hypersensitivity might not be the essential cause of neurodegeneration in CS.
Department of Pediatrics, Chiba University School of Medicine, Japan.
K
K
Sugita
J
J
Takanashi
N
N
Suzuki
H
H
Niimi
D000293:Adolescent; D016923:Cell Death / Q000528:radiation effects; D002478:Cells, Cultured; D002648:Child; D002675:Child, Preschool; D003057:Cockayne Syndrome / Q000473:pathology* / Q000523:psychology; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000473:pathology*; D009474:Neurons / Q000528:radiation effects*; D011597:Psychomotor Performance / Q000502:physiology*; D014466:Ultraviolet Rays; D014965:X-Rays