DNA sequence analysis and Igblast alignments of all 29 anti-ADAMTS13–specific mAbs grouped according to their consensus CDR3 motifs
| Patient . | Clones . | Heavy chain . | Light chain . | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Subclass . | V-gene . | D-gene . | J-gene . | CDR3 . | R:S ratio . | Homology . | κ/λ . | V-gene . | J-gene . | CDR3 . | R:S ratio . | Homology . | ||||
| FR . | CDR . | FR . | CDR . | |||||||||||||
| A | 1a | IgG4 | IGVH1-3*01 | IGHD2-15*01 | IGHJ6*04 | 17 | 1.2 | 2.2 | 86.5 | κ | IGKV4-1*01 | IGKJ4*01 | 12 | 3.2 | 3.0 | 89.5 |
| A | 1e | IgG4 | IGHD3-16*02 | IGHJ6*04 | 17 | 2.0 | 5.0 | 84.7 | κ | IGKV1-5*02 | IGKJ5*01 | 10 | 1.0 | 1.8 | 88.4 | |
| A | 1f | IgG4 | IGHD2-15*01 | IGHJ6*04 | 18 | 1.9 | 2.4 | 81.6 | κ | IGKV3-20*02 | IGKJ4*01 | 10 | 9.0 | 8.0 | 89.5 | |
| A | 1g | IgG4 | IGHD5-12*01 | IGHJ6*04 | 18 | 1.4 | 1.2 | 81.6 | κ | IGKV1-5*02 | IGKJ5*01 | 10 | 2.1 | 2.6 | 87.5 | |
| A | 2c | IgG4 | IGHD4-17*01 | IGHJ1*01 | 18 | 1.8 | 2.4 | 86.1 | λ | IGLV3-21*02 | IGLJ6*01 | 12 | 1.5 | 2.6 | 90.0 | |
| A | A-3 | IgG1 | IGHD5-12*01 | IGHJ6*03 | 18 | 1.1 | 2.4 | 84.7 | λ | IGLV1-51*02 | IGLJ6*01 | 12 | 2.1 | 2.5 | 91.1 | |
| A | A-6 | IgG1 | IGHD5-12*01 | IGHJ6*04 | 18 | 4.0 | 2.2 | 85.7 | λ | IGLV3-1*01 | IGLJ1*01 | 14 | 2.0 | 2.5 | 91.1 | |
| B | B-7 | IgG1 | IGHD5-12*01 | IGHJ6*03 | 17 | 3.4 | 7.0 | 84.6 | λ | IGLV1-51*02 | IGLJ6*01 | 14 | 2.8 | 4.0 | 89.1 | |
| A | 2a | IgG4 | IGVH1-3*01 | IGHD4-17*01 | IGHJ6*04 | 17 | 0.9 | 1.2 | 85.7 | λ | IGLV5-52*01 | IGLJ5*02 | 12 | 4.3 | 3.0 | 88.6 |
| A | 2j | IgG4 | IGHD5-12*01 | IGHJ1*01 | 17 | 0.9 | 1.3 | 85.7 | λ | IGLV3-21*02 | IGLJ6*01 | 11 | 3.8 | 4.0 | 87.5 | |
| A | A-1 | IgG4 | IGHD5-12*01 | IGHJ6*04 | 18 | 0.9 | 2.2 | 86.4 | λ | IGLV1-51*02 | IGLJ6*01 | 12 | 2.4 | 4.0 | 90.5 | |
| A | A-2 | IgG4 | IGHD5-12*01 | IGHJ6*04 | 18 | 2.0 | 2.2 | 85.7 | λ | IGLV1-51*02 | IGLJ6*01 | 14 | 2.1 | 4.0 | 89.2 | |
| B | 4a | IgG4 | IGHD2-15*01 | IGHJ6*04 | 18 | 1.3 | 2.0 | 86.4 | λ | IGLV5-52*01 | IGLJ7*01 | 9 | 4.2 | 4.3 | 87.6 | |
| B | B-1 | IgG4 | IGHD5-12*01 | IGHJ6*03 | 18 | 2.3 | 2.5 | 84.7 | λ | IGLV3-1*01 | IGLJ1*01 | 13 | 2.1 | 4.0 | 87.1 | |
| B | B-3 | IgG1 | IGHD5-12*01 | IGHJ6*03 | 19 | 3.2 | 4.3 | 84.5 | λ | IGLV1-51*02 | IGLJ6*01 | 12 | 3.0 | 3.0 | 89.2 | |
| B | B-4 | IgG1 | IGHD5-12*01 | IGHJ6*03 | 19 | 4.5 | 4.3 | 84.7 | λ | IGLV3-1*01 | IGLJ1*01 | 13 | 2.7 | 3.0 | 88.4 | |
| A | 1d | IgG4 | IGVH4-28*01 | IGHD4-17*01 | IGHJ4*02 | 11 | 0.6 | 4.0 | 90.9 | κ | IGKV4-1*01 | IGKJ2*04 | 12 | 1.5 | 2.3 | 87.9 |
| A | 1h | IgG4 | IGHD4-17*01 | IGHJ6*02 | 11 | 1.2 | 5.0 | 89.5 | κ | IGKV4-1*01 | IGKJ5*01 | 10 | 1.7 | 1.5 | 84.9 | |
| A | 2h | IgG4 | IGHD4-17*01 | IGHJ6*04 | 11 | 1.1 | 5.0 | 90.9 | λ | IGLV3-21*02 | IGLJ5*01 | 10 | 2.2 | 2.5 | 84.7 | |
| A | 2i | IgG4 | IGHD4-17*01 | IGHJ4*02 | 11 | 1.0 | 5.0 | 91.1 | λ | IGLV5-52*01 | IGLJ5*02 | 11 | 2.1 | 2.5 | 89.7 | |
| B | 3c | IgG4 | IGHD5-12*01 | IGHJ1*01 | 10 | 1.2 | 7.0 | 89.9 | κ | IGKV4-1*01 | IGKJ3*01 | 8 | 2.6 | 2.0 | 90.5 | |
| B | 3h | IgG4 | IGHD4-17*01 | IGHJ1*01 | 10 | 0.8 | 0.5 | 90.1 | κ | IGKV1-5*03 | IGKJ4*02 | 8 | 3.3 | 3.0 | 92.1 | |
| A | A-5 | IgG4 | IGVH3-30*01 | IGHD5-12*01 | IGHJ6*02 | 17 | 2.8 | 3.0 | 91.7 | λ | IGLV1-51*02 | IGLJ6*01 | 14 | 2.0 | 3.0 | 91.6 |
| B | B-8 | IgG1 | IGHD5-12*01 | IGHJ6*02 | 17 | 1.4 | 3.0 | 86.7 | λ | IGLV3-1*01 | IGLJ1*01 | 10 | 2.2 | 3.0 | 91.5 | |
| B | B-2 | IgG4 | IGVH1-69*01 | IGHD6-6*01 | IGHJ4*02 | 12 | 3.6 | 2.0 | 93.2 | λ | IGLV3-1*01 | IGLJ6*01 | 13 | 1.2 | 1.5 | 86.3 |
| B | B-6 | IgG1 | IGHD5-12*01 | IGHJ6*02 | 12 | 0.7 | 1.5 | 93.6 | λ | IGLV3-1*01 | IGLJ1*01 | 13 | 1.7 | 3.0 | 90.3 | |
| B | B-5 | IgG1 | IGVH1-69*12 | IGHD2-21*02 | IGHJ6*01 | 13 | 2.5 | 3.0 | 95.8 | λ | IGLV3-1*01 | IGLJ1*01 | 12 | 2.4 | 4.0 | 91 |
| B | 3b | IgG4 | IGVH1-69*03 | IGHD3-16*02 | IGHJ6*02 | 17 | 4.0 | 3.0 | 97.1 | κ | IGKV1-5*03 | IGKJ4*02 | 9 | 5.0 | 3.0 | 95.1 |
| B | 3j | IgG4 | IGVH1-69*06 | IGHD2-21*02 | IGHJ4*02 | 8 | 2.0 | 3.0 | 93.2 | κ | IGKV4-1*01 | IGKJ5*01 | 10 | 1.8 | 2.0 | 90.1 |
| Patient . | Clones . | Heavy chain . | Light chain . | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Subclass . | V-gene . | D-gene . | J-gene . | CDR3 . | R:S ratio . | Homology . | κ/λ . | V-gene . | J-gene . | CDR3 . | R:S ratio . | Homology . | ||||
| FR . | CDR . | FR . | CDR . | |||||||||||||
| A | 1a | IgG4 | IGVH1-3*01 | IGHD2-15*01 | IGHJ6*04 | 17 | 1.2 | 2.2 | 86.5 | κ | IGKV4-1*01 | IGKJ4*01 | 12 | 3.2 | 3.0 | 89.5 |
| A | 1e | IgG4 | IGHD3-16*02 | IGHJ6*04 | 17 | 2.0 | 5.0 | 84.7 | κ | IGKV1-5*02 | IGKJ5*01 | 10 | 1.0 | 1.8 | 88.4 | |
| A | 1f | IgG4 | IGHD2-15*01 | IGHJ6*04 | 18 | 1.9 | 2.4 | 81.6 | κ | IGKV3-20*02 | IGKJ4*01 | 10 | 9.0 | 8.0 | 89.5 | |
| A | 1g | IgG4 | IGHD5-12*01 | IGHJ6*04 | 18 | 1.4 | 1.2 | 81.6 | κ | IGKV1-5*02 | IGKJ5*01 | 10 | 2.1 | 2.6 | 87.5 | |
| A | 2c | IgG4 | IGHD4-17*01 | IGHJ1*01 | 18 | 1.8 | 2.4 | 86.1 | λ | IGLV3-21*02 | IGLJ6*01 | 12 | 1.5 | 2.6 | 90.0 | |
| A | A-3 | IgG1 | IGHD5-12*01 | IGHJ6*03 | 18 | 1.1 | 2.4 | 84.7 | λ | IGLV1-51*02 | IGLJ6*01 | 12 | 2.1 | 2.5 | 91.1 | |
| A | A-6 | IgG1 | IGHD5-12*01 | IGHJ6*04 | 18 | 4.0 | 2.2 | 85.7 | λ | IGLV3-1*01 | IGLJ1*01 | 14 | 2.0 | 2.5 | 91.1 | |
| B | B-7 | IgG1 | IGHD5-12*01 | IGHJ6*03 | 17 | 3.4 | 7.0 | 84.6 | λ | IGLV1-51*02 | IGLJ6*01 | 14 | 2.8 | 4.0 | 89.1 | |
| A | 2a | IgG4 | IGVH1-3*01 | IGHD4-17*01 | IGHJ6*04 | 17 | 0.9 | 1.2 | 85.7 | λ | IGLV5-52*01 | IGLJ5*02 | 12 | 4.3 | 3.0 | 88.6 |
| A | 2j | IgG4 | IGHD5-12*01 | IGHJ1*01 | 17 | 0.9 | 1.3 | 85.7 | λ | IGLV3-21*02 | IGLJ6*01 | 11 | 3.8 | 4.0 | 87.5 | |
| A | A-1 | IgG4 | IGHD5-12*01 | IGHJ6*04 | 18 | 0.9 | 2.2 | 86.4 | λ | IGLV1-51*02 | IGLJ6*01 | 12 | 2.4 | 4.0 | 90.5 | |
| A | A-2 | IgG4 | IGHD5-12*01 | IGHJ6*04 | 18 | 2.0 | 2.2 | 85.7 | λ | IGLV1-51*02 | IGLJ6*01 | 14 | 2.1 | 4.0 | 89.2 | |
| B | 4a | IgG4 | IGHD2-15*01 | IGHJ6*04 | 18 | 1.3 | 2.0 | 86.4 | λ | IGLV5-52*01 | IGLJ7*01 | 9 | 4.2 | 4.3 | 87.6 | |
| B | B-1 | IgG4 | IGHD5-12*01 | IGHJ6*03 | 18 | 2.3 | 2.5 | 84.7 | λ | IGLV3-1*01 | IGLJ1*01 | 13 | 2.1 | 4.0 | 87.1 | |
| B | B-3 | IgG1 | IGHD5-12*01 | IGHJ6*03 | 19 | 3.2 | 4.3 | 84.5 | λ | IGLV1-51*02 | IGLJ6*01 | 12 | 3.0 | 3.0 | 89.2 | |
| B | B-4 | IgG1 | IGHD5-12*01 | IGHJ6*03 | 19 | 4.5 | 4.3 | 84.7 | λ | IGLV3-1*01 | IGLJ1*01 | 13 | 2.7 | 3.0 | 88.4 | |
| A | 1d | IgG4 | IGVH4-28*01 | IGHD4-17*01 | IGHJ4*02 | 11 | 0.6 | 4.0 | 90.9 | κ | IGKV4-1*01 | IGKJ2*04 | 12 | 1.5 | 2.3 | 87.9 |
| A | 1h | IgG4 | IGHD4-17*01 | IGHJ6*02 | 11 | 1.2 | 5.0 | 89.5 | κ | IGKV4-1*01 | IGKJ5*01 | 10 | 1.7 | 1.5 | 84.9 | |
| A | 2h | IgG4 | IGHD4-17*01 | IGHJ6*04 | 11 | 1.1 | 5.0 | 90.9 | λ | IGLV3-21*02 | IGLJ5*01 | 10 | 2.2 | 2.5 | 84.7 | |
| A | 2i | IgG4 | IGHD4-17*01 | IGHJ4*02 | 11 | 1.0 | 5.0 | 91.1 | λ | IGLV5-52*01 | IGLJ5*02 | 11 | 2.1 | 2.5 | 89.7 | |
| B | 3c | IgG4 | IGHD5-12*01 | IGHJ1*01 | 10 | 1.2 | 7.0 | 89.9 | κ | IGKV4-1*01 | IGKJ3*01 | 8 | 2.6 | 2.0 | 90.5 | |
| B | 3h | IgG4 | IGHD4-17*01 | IGHJ1*01 | 10 | 0.8 | 0.5 | 90.1 | κ | IGKV1-5*03 | IGKJ4*02 | 8 | 3.3 | 3.0 | 92.1 | |
| A | A-5 | IgG4 | IGVH3-30*01 | IGHD5-12*01 | IGHJ6*02 | 17 | 2.8 | 3.0 | 91.7 | λ | IGLV1-51*02 | IGLJ6*01 | 14 | 2.0 | 3.0 | 91.6 |
| B | B-8 | IgG1 | IGHD5-12*01 | IGHJ6*02 | 17 | 1.4 | 3.0 | 86.7 | λ | IGLV3-1*01 | IGLJ1*01 | 10 | 2.2 | 3.0 | 91.5 | |
| B | B-2 | IgG4 | IGVH1-69*01 | IGHD6-6*01 | IGHJ4*02 | 12 | 3.6 | 2.0 | 93.2 | λ | IGLV3-1*01 | IGLJ6*01 | 13 | 1.2 | 1.5 | 86.3 |
| B | B-6 | IgG1 | IGHD5-12*01 | IGHJ6*02 | 12 | 0.7 | 1.5 | 93.6 | λ | IGLV3-1*01 | IGLJ1*01 | 13 | 1.7 | 3.0 | 90.3 | |
| B | B-5 | IgG1 | IGVH1-69*12 | IGHD2-21*02 | IGHJ6*01 | 13 | 2.5 | 3.0 | 95.8 | λ | IGLV3-1*01 | IGLJ1*01 | 12 | 2.4 | 4.0 | 91 |
| B | 3b | IgG4 | IGVH1-69*03 | IGHD3-16*02 | IGHJ6*02 | 17 | 4.0 | 3.0 | 97.1 | κ | IGKV1-5*03 | IGKJ4*02 | 9 | 5.0 | 3.0 | 95.1 |
| B | 3j | IgG4 | IGVH1-69*06 | IGHD2-21*02 | IGHJ4*02 | 8 | 2.0 | 3.0 | 93.2 | κ | IGKV4-1*01 | IGKJ5*01 | 10 | 1.8 | 2.0 | 90.1 |
Listed are the genes used for the variable H- and L-chain region, showing the V-, D-, and J-genes (Fabs and whole IgGs), the IgG subclass, the percentage of homology to the closest germ-line genes, the length of the CDR3 regions, and the ratio of the replacement (R) to silent (S) somatic hypermutation in the framework regions (FR1-3) and the CDR1 and CDR2 region. Clone identity: phage display-derived Fabs are indicated by italic letters and numbered with either 1 (pat A) or 3 (pat B) when originating from the κ library and 2 (pat A) or 4 (pat B) when originating from the λ library; whole IgGs derived from EBV-transformed switched memory B cells are labeled with capital letters.