2024-03-29T15:40:20Z
https://nagoya.repo.nii.ac.jp/oai
oai:nagoya.repo.nii.ac.jp:00003921
2023-11-16T06:58:58Z
499:508:509:511
Identification of plasma antibody epitopes and gene abnormalities in Japanese hemophilia a patients with factor VIII inhibitor
Sugihara, Takuro
Takahashi, Isao
Kojima, Tetsuhito
Okamoto, Yoshihiro
Yamamoto, Koji
Kamiya, Tadashi
Matsushita, Tadashi
Saito, Hidehiko
open access
Factor VIII
Hemophilia A
Factor VIII inhibitor
Southern blotting
Gene inversion
Eleven Japanese hemophilia A patients with anti-factor VIII (FVIII)a) inhibitors were studied to localize both their inhibitory antibody epitopes and their genotypes. The inhibitor epitopes were studied in nine severe hemophilia A patients by means of a scanning method using the oligopeptide panel covering the FVIII polypeptides without the B domain. The 107 15 mer-peptides were synthesized on solid-phase pins and analyzed for their reactivity with diluted patient plasma. As indicated previously, a series of peptides corresponding to the A2 and C2 domains were recognized by plasma antibodies from 2 patients and 4 patients, respectively. In contrast, all the antibodies bound to several epitopes in the A3 domain, while an epitope 1809-1821 covering the putative factor IX binding site was found in 3 patients. Southern blotting analysis showed that 8 out of 11 patients had either gene deletions or inversions of the FVIII gene, indicating a higher proportion of gross gene alterations in inhibitor-positive hemophilia A patients. However, the correlation of gene abnormality type with epitope location was not fully established.
Nagoya University School of Medicine
2000-05
eng
departmental bulletin paper
VoR
https://doi.org/10.18999/nagjms.63.1-2.25
http://hdl.handle.net/2237/5351
https://nagoya.repo.nii.ac.jp/records/3921
10.18999/nagjms.63.1-2.25
2186-3326
0027-7622
Nagoya Journal of Medical Science
63
1-2
25
39
https://nagoya.repo.nii.ac.jp/record/3921/files/nj6314.pdf
application/pdf
267.3 kB
2018-02-19