Hypersensitivity to ionizing radiation in cultured cells from Down syndrome patients

Topic: Brain proteins, microglia and lab models

Down syndrome is caused by trisomy of chromosome 21 and is comprised of a constellation of abnormalities including neuropathological features that closely resemble those characterizing the neurodegeneration of Alzheimer disease. Because cultured cell lines from patients with Alzheimer disease and other neurodegenerations have a hypersensitivity to the lethal effects of DNA-damaging agents, we studied the response of Down syndrome lymphoblastoid lines to the lethal effects of ionizing and ultraviolet radiation. Lines from the four Down syndrome patients were more sensitive to X-rays than lines from 28 normal donors (P = 10(-4)), while survival of the Down syndrome lines after ultraviolet irradiation was not significantly different from normal. This hypersensitivity to X-rays, which may reflect defective repair of X-ray-induced DNA damage, represents the first abnormality common to cultured cells from both Down syndrome and Alzheimer disease patients.

@article{otsuka1985-3159854,
  author  = {Otsuka, F. and Tarone, R. E. and Seguin, L. R. and Robbins, J. H.},
  title   = {{Hypersensitivity to ionizing radiation in cultured cells from Down syndrome patients}},
  journal = {Journal of the neurological sciences},
  year    = {1985},
  doi     = {10.1016/0022-510x(85)90011-5},
  pmid    = {3159854},
  url     = {https://doi.org/10.1016/0022-510x(85)90011-5}
}

Key findings

• Lines from the four Down syndrome patients were more sensitive to X-rays than lines from 28 normal donors (P = 10(-4)), while survival of the Down syndrome lines after ultraviolet irradiation was not significantly different from normal.

Why it matters

Cells from four Down syndrome patients were more sensitive to X-rays than cells from normal donors, which may reflect defective repair of X-ray-induced DNA damage. This is a laboratory cell study and does not test any treatment, cure, or prevention for Alzheimer's disease.

Study details

• Evidence strengthLow

Read the original

• DOI: 10.1016/0022-510x(85)90011-5
• PubMed: 3159854
• Original source →

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Studies this one cites

Referenced by this study and also in the alzhub library, so you can read them here.

• Neurofibrillary tangles, granulovacuolar degeneration, and neuron loss in Down Syndrome: quantitative comparison with Alzheimer dementia Annals of neurology · 1980-05-01 · usefulness score 43/100 · Down syndrome and Alzheimer's

Later studies that cite this one

1 study in the alzhub library lists this one in its references.

• Fluorescent light-induced chromatid breaks distinguish Alzheimer disease cells from normal cells in tissue culture Proceedings of the National Academy of Sciences of the United States of America · 1996-05-01 · usefulness score 36/100 · Down syndrome and Alzheimer's

Related studies in Down syndrome and Alzheimer's

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• Study of spindle microtubule reassembly in cells from Alzheimer and Down syndrome patients following exposure to colcemid Alzheimer disease and associated disorders · 1990-01-01 · usefulness score 30/100
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Also tagged “down”

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• Oxidative stress and mitochondrial dysfunction in Down syndrome Advances in experimental medicine and biology · 2012-01-01 · usefulness score 41/100 · Down syndrome and Alzheimer's
• Age-associated chromosome 21 loss in Down syndrome: possible relevance to mosaicism and Alzheimer disease American journal of medical genetics · 1993-03-01 · usefulness score 34/100 · Down syndrome and Alzheimer's
• Quantification of modified amyloid beta peptides in Alzheimer disease and Down syndrome brains Journal of neuropathology and experimental neurology · 1998-11-01 · usefulness score 40/100 · Down syndrome and Alzheimer's

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