J Cell Physiol. 1979 Nov;101(2):311-23. doi: 10.1002/jcp.1041010211.

Specific cellular defects in patients with Fanconi anemia.

Journal of cellular physiology

R Weksberg, M Buchwald, P Sargent, M W Thompson, L Siminovitch

PMID: 511954 DOI: 10.1002/jcp.1041010211

Abstract

Measurements of plating efficiency, accumulation of metaphases and generation times have shown that fibroblast from patients with Fanconi anemia (FA) have decreased probability of completing a further division after successful mitosis. Thus FA cells show decreased growth rates and increased generation times. We have also measured the survival of FA fibroblasts and lymphoblasts after treatment with a variety of mutagens. All FA cells show an increased sensitivity to drugs such as MMC and psoralen plus long wave length UV which cause DNA interstrand crosslinks. FA strains show varying degrees of sensitivity to these drugs and the extent of this sensitivity seems to be characteristic of each patient. FA cells are equal to controls in their sensitivity to other alkylating agents such as ethyl methane sulfonate, N-methyl-N1-nitro-N-nitrosoguanidine and actinomycin D. Both the decreased growth and increased drug sensitivity may result from defect in DNA replication or repair.

Cited by

Substances

• Mitomycins
• Ficusin

MeSH terms

• Adolescent
• Adult
• Anemia, Aplastic / pathology
• Cell Division
• Child
• Fanconi Anemia / pathology
• Female
• Fibroblasts / cytology
• Fibroblasts / drug effects
• Fibroblasts / pathology
• Fibroblasts / radiation effects
• Ficusin / pharmacology
• Humans
• Karyotyping
• Male
• Metaphase / drug effects
• Mitomycins / pharmacology
• Mutation
• Photochemotherapy / adverse effects
• Ultraviolet Rays

Publication Types

• Journal Article
• Research Support, U.S. Gov't, P.H.S.

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