Table 1 Molecular characteristics of the human MuSK autoantibodies. The molecular characteristics of the human MuSK-specific mAbs 2E6, 6C6 and the clonal variants of 2E6. The replacement mutations in the variable region gene segment were counted from the beginning of framework 1 through the invariable cysteine at position 104. The mutations in the CDR3 were counted between cysteine 104 and the invariable tryptophan (W) or phenylalanine (F) at position 118 in both the heavy chain and the light chain, respectively. No FR4 mutations were observed. N-linked glycosylation motif (N-X-S/T; X is any AA except proline).
Name of IgG4 Clone (mAb) | MG PatientID | B cell subset phenotype (by index sorting) | Isotype and IgG subclass | Variable region family and allele | Joining region family and allele | Diversity region family and allele | Amino acid replacements in variable region gene segment | Amino acid replacements in CDR3 | CDR3 length (NT) | N-linked glycosylation site In variable region |
|---|---|---|---|---|---|---|---|---|---|---|
2E6 | MuSK MG-1 | Plasmablast | IgG4 | IGHV4-61*01,IGHV4-61*08 | IGHJ6*03 F (b) | IGHD3-3*01 F | 23 + 2 insertions | 4 | 78 | 1 acquired in FR1 |
| Â | Â | Â | 2E6 CVA (IgG) | IGHV4-61*01,IGHV4-61*08 | IGHJ6*03 F | IGHD3-10*01 F | 17 + 2 insertions | 3 | 78 | 1 acquired in FR1 |
| Â | Â | Â | 2E6 CVB (IgG4) | IGHV4-61*01,IGHV4-61*08 | IGHJ6*03 F | IGHD3-3*01 F | 17 + 2 insertions | 5 | 78 | 1 acquired in FR1 |
| Â | Â | Â | 2E6 CVC (IgG4) and 2E6 CVC (IgG) | IGHV4-61*01,IGHV4-61*08 | IGHJ6*03 F | IGHD3-3*01 F | 24 + 2 insertions | 4 | 78 | 1 acquired in FR1 |
|  |  |  | κ | IGKV3-20*01 F | IGKJ3*01 F (a) | – | 9 | 1 | 33 | – |
6C6 | MuSK MG-4 | Plasmablast | IgG4 | IGHV3-11*01 F | IGHJ4*02 F | IGHD3-10*01 F | 14 | 3 | 39 | – |
|  |  |  | κ | IGKV3-11*01 F | IGKJ4*01 F | – | 14 | 2 | 39 | 1 acquired CDR2/FR2 |