Koopmans defect

Mutations of the Bj3 chain are less common. Substitution of Cys for Gly at amino acid 15 results in prolonged polymerization from delayed release of fibrinopeptide B (Yoshida et al., 1991). Mutation of Ala68 to Thr results in defective binding of thrombin to fibrin and consequent thrombosis (Koopman et al., 1992). Deletion of residues 9-72, which includes a cysteine that is normally part of a disulfide bond, results in delayed release of both fibrinopeptides A and B (Liu et al., 1985). 

Koopman, J., Haverkate, F., Lord, S. T., Grimbergen, J., and Mannucci, P. M. (1992). Molecular basis of fibrinogen Naples associated with defective thrombin binding and thrombophilia. Homozygous substitution of B beta 68 Ala —> Thr. J. Clin. Invest. 90, 238-244. 

Ultraviolet photoelectron spectroscopy combined with ab initio calculations of the five- and six-membered unsaturated rings containing phosphorus and arsenic have been studied <1995JST57>. Assigning the spectra, no defect of Koopmans theorem has been found in the low-ionization-energy region. Reorganization and correlation effects should be comparable and be considered free from any defects of Koopmans theorem. 

---