FANCD2 monoubiquitination is normal in other inherited bone marrow failure and chromosomal instability syndromes.

(A) EBV-immortalized lymphocytes from patients with the indicated bone marrow failure syndromes were harvested before or 6 hours after treatment with 10 Gy radiation. Cell lysates were analyzed by immunoblotting with an anti-FANCD2 FI17 monoclonal antibody. FA indicates Fanconi anemia; SCN, severe congenital neutropenia; SDS, Shwachman-Diamond syndrome; DKC, dyskeratosis congenita; and DBA, Diamond-Blackfan anemia. (B) EBV-immortalized lymphocytes from patients with the indicated chromosomal breakage syndromes were harvested before or 6 hours after treatment with 10 Gy radiation. Cell lysates were analyzed by immunoblotting with an anti-FANCD2 FI17 monoclonal antibody. CS indicates Cockayne syndrome; WS, Werner syndrome; NBS, Nijmegen breakage syndrome; BS, Bloom syndrome; and XP, xeroderma pigmentosa.