TCRBV repertoire in 9 patients with A-T
CD4 . | CD8 . | |||||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Vβ . | Normal range (median) . | 1 . | 2 . | 3 . | 4 . | 5 . | 6 . | 7 . | 8 . | 9 . | Normal range (median) . | 1 . | 2 . | 3 . | 4 . | 5 . | 6 . | 7 . | 8 . | 9 . |
1 | 2.1-4.0 (3.4) | 2.4 | 2.6 | 1.7 | 3.0 | 2.0 | 2.5 | 2.3 | 2.6 | 3.4 | 2.5-7.9 (3.8) | 0.9 | 4.9 | 0.5 | 5.6 | 0.9 | 1.4 | 4.0 | 1.3 | 1.9 |
2 | 3.8-13.1 (9.6) | 0.5 | 2.6 | 5.5 | 7.8 | 7.5 | 3.0 | 4.0 | 5.7 | 8.0 | 0.7-20.5 (5.6) | 0.1 | 0.5 | 2.8 | 3.3 | 0.8 | 0.6 | 17.0 | 1.3 | 0.9 |
3 | 0.6-9.8 (4.1) | 5.2 | 4.3 | 2.3 | 6.5 | 4.1 | 3.1 | 1.4 | 0.5 | 2.0 | 0.1-19.3 (3.7) | 29.3 | 5.0 | 7.7 | 8.2 | 7.8 | 3.7 | 4.0 | 10.6 | 3.7 |
4 | 1.5-2.5 (1.8) | ND | 0.8 | ND | 1.4 | ND | ND | 0 | ND | 0 | 0.3-2.0 (1.4) | ND | 0.5 | ND | 0.4 | ND | ND | 0 | ND | 0 |
5.1 | 3.8-8.6 (6.1) | 6.5 | 9.2 | 4.7 | 10.8 | 5.9 | 15.6 | 5.0 | 7.2 | 8.9 | 1.2-6.1 (2.7) | 0.8 | 3.0 | 0.3 | 2.0 | 1.2 | 2.8 | 3.7 | 1.4 | 1.2 |
5.2 | 0.1-2.7 (0.8) | 0.7 | 0.8 | 1.1 | 1.3 | 0.4 | 0.7 | 0.4 | 1.0 | 1.5 | 0.2-4.0 (0.8) | 0.6 | 0.3 | 0.2 | 3.8 | 0.2 | 0.6 | 0.9 | 0.2 | 0.8 |
5.3 | 0.1-3.4 (1.8) | 1.0 | 1.0 | 0.7 | 0.8 | 1.2 | 0.9 | 0.3 | 1.0 | 1.2 | 0.4-21.6 (1.5) | 1.1 | 0.6 | 0.1 | 0 | <0.1 | 0.5 | 0.2 | 0.6 | 0.1 |
7.1 | 1.8-2.8 (2.0) | ND | 1.1 | ND | 0.8 | ND | ND | 0.8 | 1.0 | 5.0 | 1.9-4.6 (3.7) | ND | 14.3 | ND | 1.5 | ND | ND | 6.5 | 3.4 | 1.3 |
7.2 | 0.1-1.7 (0.9) | 2.6 | 1.5 | 0.6 | 1.2 | 1.6 | 0.6 | <0.1 | ND | 1.5 | 0.1-5.7 (0.9) | 7.6 | 1.0 | 8.9 | 0.4 | 0.1 | 2.6 | <0.1 | ND | 47 |
8 | 3.2-10.0 (4.9) | 7.5 | 4.4 | 5.8 | 5.6 | 3.2 | 2.0 | 1.9 | 3.6 | 3.3 | 1.0-12.4 (3.2) | 1.8 | 5.5 | <0.1 | 2.3 | 0.5 | 2.8 | 2.0 | 1.4 | 0.6 |
9 | 2.5-14.5 (3.8) | 7.0 | 3.9 | 3.7 | 2.4 | 3.7 | 3.0 | 1.7 | 2.6 | 3.3 | 1.2-5.2 (2.3) | 2.6 | 1.0 | 1.0 | 0.4 | 0.8 | 1.8 | 2.2 | 1.0 | 1.5 |
11 | 0.5-1.1 (0.8) | 2.3 | 0.9 | 1.6 | 0.5 | 0.4 | 0.5 | 0.7 | 3.2 | 0.8 | 0.4-2.0 (0.6) | 0.8 | 0.4 | 0.2 | 0.4 | 2.8 | 0.5 | 1.3 | 2.2 | 0.1 |
12 | 0.7-3.3 (1.8) | 1.2 | 4 | 1.6 | 4 | 2.6 | 1.8 | 2.3 | 2.4 | 2.7 | 0.3-4.5 (1.3) | 1.5 | 3.1 | 0.3 | 5.3 | <0.1 | 1.4 | 1.3 | 1.4 | 0.7 |
13.1 | 1.0-4.5 (3.6) | 4.6 | 4.5 | 3.9 | 3.3 | 3.1 | 3.3 | 1.8 | 3.1 | 4.4 | 1.1-7.1 (3.4) | 0.9 | 13.8 | 2.4 | 1.4 | 1.1 | 2.3 | 3.0 | 2.2 | 5.6 |
13.2 | 1.4-4.0 (2.8) | ND | 2.3 | ND | 2.6 | ND | ND | 0.2 | ND | 1.3 | 1.1-6.3 (2.8) | ND | 1.6 | ND | 2 | ND | ND | 0.4 | ND | 0.4 |
13.6 | 0.8-4.3 (2.0) | 5.0 | 1.6 | 4.8 | 1.3 | 2.4 | 2.7 | 1.2 | 1.9 | 2.8 | 0.1-3.6 (1.5) | 2.9 | 0.3 | 1.6 | 6.0 | 1.1 | 0.4 | 0.5 | 0.8 | 0.6 |
14 | 1.0-3.5 (2.6) | 8.0 | 2.2 | 5.0 | 2.4 | 2.0 | 1.1 | 1.9 | 2.5 | 4.8 | 2.1-10.6 (4.2) | 10 | 2.1 | 5.3 | 5.7 | 1.0 | 5.2 | 3.0 | 4.5 | 2.7 |
16 | 0.7-3.8 (1.2) | 2.7 | 3.0 | 0.7 | 4.2 | 0.9 | 0.4 | 1.0 | 0.8 | 2.3 | 0.1-3.8 (0.9) | 0.4 | 3.2 | <0.1 | 2.8 | 0.1 | 0.8 | 1.1 | 6.2 | 3.8 |
17 | 2.6-8.2 (5.5) | 2.2 | 4.5 | 3.2 | 6.0 | 11.5 | 2.6 | 2.4 | 4.1 | 4.5 | 1.7-23.2 (4.7) | 5.4 | 13 | 6.4 | 2.0 | 0.2 | 16.5 | 2.0 | 10.6 | 1.7 |
18 | 0.4-4.3 (1.7) | 0.9 | 1.2 | 0.4 | 1.1 | 1.4 | 1.5 | 0.5 | 1.9 | 1.1 | 0.1-4.1 (0.6) | 0.3 | <0.1 | <0.1 | <0.1 | 0.6 | <0.1 | 0.2 | 1.2 | 0.4 |
20 | 1.9-3.9 (2.7) | 4.1 | 0 | 3.9 | 0 | 1.4 | 2.0 | 1.6 | 3.1 | 1.7 | 0.6-3.1 (1.8) | 2.0 | 0 | 1.1 | 0 | 0.5 | 1.7 | 1.2 | 1.3 | 0.9 |
21.3 | 1.6-3.0 (2.3) | 2.6 | 1.8 | 1.8 | 4.4 | 1.1 | 0.7 | 1.2 | 1.6 | 2.0 | 1.0-5.6 (1.7) | 1.0 | 2.3 | 0.2 | 1.0 | <0.1 | 0.9 | 3.2 | 1.1 | 0.6 |
22 | 2.4-9.8 (4.1) | 4.9 | 2.4 | 3.1 | 3.0 | 2.6 | 5.6 | 1.8 | 0.8 | 3.8 | 0.7-6.7 (2.9) | 0.4 | 3.9 | 4.3 | 0.4 | 0.4 | 1.5 | 1.6 | 0.1 | 1.2 |
23 | 0.1-1.2 (0.4) | 1.7 | 1.4 | 1.4 | 0.4 | <0.1 | 0.9 | 0.2 | 0.5 | 0.7 | 0.1-18.9 (1.1) | 0.6 | 1.2 | 0.3 | 1.6 | 0.7 | 1.2 | 1.2 | 0.7 | 0.9 |
Total | 70.7 | 73.7 | 61.3 | 56.5 | 74.6 | 58.2 | 54.4 | 34.6 | 51 | 70.6 | 57.1 | 70.3 | 81.3 | 43.5 | 56.4 | 20.4 | 49.2 | 60.5 | 53.4 | 78.6 |
CD4 . | CD8 . | |||||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Vβ . | Normal range (median) . | 1 . | 2 . | 3 . | 4 . | 5 . | 6 . | 7 . | 8 . | 9 . | Normal range (median) . | 1 . | 2 . | 3 . | 4 . | 5 . | 6 . | 7 . | 8 . | 9 . |
1 | 2.1-4.0 (3.4) | 2.4 | 2.6 | 1.7 | 3.0 | 2.0 | 2.5 | 2.3 | 2.6 | 3.4 | 2.5-7.9 (3.8) | 0.9 | 4.9 | 0.5 | 5.6 | 0.9 | 1.4 | 4.0 | 1.3 | 1.9 |
2 | 3.8-13.1 (9.6) | 0.5 | 2.6 | 5.5 | 7.8 | 7.5 | 3.0 | 4.0 | 5.7 | 8.0 | 0.7-20.5 (5.6) | 0.1 | 0.5 | 2.8 | 3.3 | 0.8 | 0.6 | 17.0 | 1.3 | 0.9 |
3 | 0.6-9.8 (4.1) | 5.2 | 4.3 | 2.3 | 6.5 | 4.1 | 3.1 | 1.4 | 0.5 | 2.0 | 0.1-19.3 (3.7) | 29.3 | 5.0 | 7.7 | 8.2 | 7.8 | 3.7 | 4.0 | 10.6 | 3.7 |
4 | 1.5-2.5 (1.8) | ND | 0.8 | ND | 1.4 | ND | ND | 0 | ND | 0 | 0.3-2.0 (1.4) | ND | 0.5 | ND | 0.4 | ND | ND | 0 | ND | 0 |
5.1 | 3.8-8.6 (6.1) | 6.5 | 9.2 | 4.7 | 10.8 | 5.9 | 15.6 | 5.0 | 7.2 | 8.9 | 1.2-6.1 (2.7) | 0.8 | 3.0 | 0.3 | 2.0 | 1.2 | 2.8 | 3.7 | 1.4 | 1.2 |
5.2 | 0.1-2.7 (0.8) | 0.7 | 0.8 | 1.1 | 1.3 | 0.4 | 0.7 | 0.4 | 1.0 | 1.5 | 0.2-4.0 (0.8) | 0.6 | 0.3 | 0.2 | 3.8 | 0.2 | 0.6 | 0.9 | 0.2 | 0.8 |
5.3 | 0.1-3.4 (1.8) | 1.0 | 1.0 | 0.7 | 0.8 | 1.2 | 0.9 | 0.3 | 1.0 | 1.2 | 0.4-21.6 (1.5) | 1.1 | 0.6 | 0.1 | 0 | <0.1 | 0.5 | 0.2 | 0.6 | 0.1 |
7.1 | 1.8-2.8 (2.0) | ND | 1.1 | ND | 0.8 | ND | ND | 0.8 | 1.0 | 5.0 | 1.9-4.6 (3.7) | ND | 14.3 | ND | 1.5 | ND | ND | 6.5 | 3.4 | 1.3 |
7.2 | 0.1-1.7 (0.9) | 2.6 | 1.5 | 0.6 | 1.2 | 1.6 | 0.6 | <0.1 | ND | 1.5 | 0.1-5.7 (0.9) | 7.6 | 1.0 | 8.9 | 0.4 | 0.1 | 2.6 | <0.1 | ND | 47 |
8 | 3.2-10.0 (4.9) | 7.5 | 4.4 | 5.8 | 5.6 | 3.2 | 2.0 | 1.9 | 3.6 | 3.3 | 1.0-12.4 (3.2) | 1.8 | 5.5 | <0.1 | 2.3 | 0.5 | 2.8 | 2.0 | 1.4 | 0.6 |
9 | 2.5-14.5 (3.8) | 7.0 | 3.9 | 3.7 | 2.4 | 3.7 | 3.0 | 1.7 | 2.6 | 3.3 | 1.2-5.2 (2.3) | 2.6 | 1.0 | 1.0 | 0.4 | 0.8 | 1.8 | 2.2 | 1.0 | 1.5 |
11 | 0.5-1.1 (0.8) | 2.3 | 0.9 | 1.6 | 0.5 | 0.4 | 0.5 | 0.7 | 3.2 | 0.8 | 0.4-2.0 (0.6) | 0.8 | 0.4 | 0.2 | 0.4 | 2.8 | 0.5 | 1.3 | 2.2 | 0.1 |
12 | 0.7-3.3 (1.8) | 1.2 | 4 | 1.6 | 4 | 2.6 | 1.8 | 2.3 | 2.4 | 2.7 | 0.3-4.5 (1.3) | 1.5 | 3.1 | 0.3 | 5.3 | <0.1 | 1.4 | 1.3 | 1.4 | 0.7 |
13.1 | 1.0-4.5 (3.6) | 4.6 | 4.5 | 3.9 | 3.3 | 3.1 | 3.3 | 1.8 | 3.1 | 4.4 | 1.1-7.1 (3.4) | 0.9 | 13.8 | 2.4 | 1.4 | 1.1 | 2.3 | 3.0 | 2.2 | 5.6 |
13.2 | 1.4-4.0 (2.8) | ND | 2.3 | ND | 2.6 | ND | ND | 0.2 | ND | 1.3 | 1.1-6.3 (2.8) | ND | 1.6 | ND | 2 | ND | ND | 0.4 | ND | 0.4 |
13.6 | 0.8-4.3 (2.0) | 5.0 | 1.6 | 4.8 | 1.3 | 2.4 | 2.7 | 1.2 | 1.9 | 2.8 | 0.1-3.6 (1.5) | 2.9 | 0.3 | 1.6 | 6.0 | 1.1 | 0.4 | 0.5 | 0.8 | 0.6 |
14 | 1.0-3.5 (2.6) | 8.0 | 2.2 | 5.0 | 2.4 | 2.0 | 1.1 | 1.9 | 2.5 | 4.8 | 2.1-10.6 (4.2) | 10 | 2.1 | 5.3 | 5.7 | 1.0 | 5.2 | 3.0 | 4.5 | 2.7 |
16 | 0.7-3.8 (1.2) | 2.7 | 3.0 | 0.7 | 4.2 | 0.9 | 0.4 | 1.0 | 0.8 | 2.3 | 0.1-3.8 (0.9) | 0.4 | 3.2 | <0.1 | 2.8 | 0.1 | 0.8 | 1.1 | 6.2 | 3.8 |
17 | 2.6-8.2 (5.5) | 2.2 | 4.5 | 3.2 | 6.0 | 11.5 | 2.6 | 2.4 | 4.1 | 4.5 | 1.7-23.2 (4.7) | 5.4 | 13 | 6.4 | 2.0 | 0.2 | 16.5 | 2.0 | 10.6 | 1.7 |
18 | 0.4-4.3 (1.7) | 0.9 | 1.2 | 0.4 | 1.1 | 1.4 | 1.5 | 0.5 | 1.9 | 1.1 | 0.1-4.1 (0.6) | 0.3 | <0.1 | <0.1 | <0.1 | 0.6 | <0.1 | 0.2 | 1.2 | 0.4 |
20 | 1.9-3.9 (2.7) | 4.1 | 0 | 3.9 | 0 | 1.4 | 2.0 | 1.6 | 3.1 | 1.7 | 0.6-3.1 (1.8) | 2.0 | 0 | 1.1 | 0 | 0.5 | 1.7 | 1.2 | 1.3 | 0.9 |
21.3 | 1.6-3.0 (2.3) | 2.6 | 1.8 | 1.8 | 4.4 | 1.1 | 0.7 | 1.2 | 1.6 | 2.0 | 1.0-5.6 (1.7) | 1.0 | 2.3 | 0.2 | 1.0 | <0.1 | 0.9 | 3.2 | 1.1 | 0.6 |
22 | 2.4-9.8 (4.1) | 4.9 | 2.4 | 3.1 | 3.0 | 2.6 | 5.6 | 1.8 | 0.8 | 3.8 | 0.7-6.7 (2.9) | 0.4 | 3.9 | 4.3 | 0.4 | 0.4 | 1.5 | 1.6 | 0.1 | 1.2 |
23 | 0.1-1.2 (0.4) | 1.7 | 1.4 | 1.4 | 0.4 | <0.1 | 0.9 | 0.2 | 0.5 | 0.7 | 0.1-18.9 (1.1) | 0.6 | 1.2 | 0.3 | 1.6 | 0.7 | 1.2 | 1.2 | 0.7 | 0.9 |
Total | 70.7 | 73.7 | 61.3 | 56.5 | 74.6 | 58.2 | 54.4 | 34.6 | 51 | 70.6 | 57.1 | 70.3 | 81.3 | 43.5 | 56.4 | 20.4 | 49.2 | 60.5 | 53.4 | 78.6 |
Analyses were done with Vβ-specific mAbs; the nomenclature adopted is from Wei et al.52 Results are the percentages of TCRαβ T cells expressing the indicated TCRBV gene.
ND indicates determinations not done; shading, underexpressed TCRBV genes; and italics, overexpressed TCRBVs.