The Response of Cockayne Syndrome Cells to UV-Irradiation
Cockayne syndrome (CS) is a rare autosomal recessive disorder. The principal clinical features are dwarfism with a small head and senile appearance, skeletal abnormalities, loss of adipose tissue, severe mental retardation, retinal degeneration and severe photosensitivity. The onset occurs after several months of apparently normal development. The retinal, neurological and mental symptoms are progressive, as is the loss of adipose tissue1. There are no reports in the literature of elevated frequencies of cancer, as found for example in xeroderma pigmentosum. With one or two exceptions the karyotype is normal.
KeywordsRetinal Degeneration Xeroderma Pigmentosum Complementation Group Severe Mental Retardation Defective Cell
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