Table 1.
Commercial antibodies for the diagnosis of muscular dystrophies.
| Antigen | Host/Isotype | Clone | IHC | WB |
|---|---|---|---|---|
| Spectrin | Mouse IgG2b | RBC2/3D5 | Y | N |
| Dystrophin (N term) | Mouse IgG2a | Dy10/12B2 | Y | Y |
| Dystrophin (rod domain) | Mouse IgG2a | Dy4/6D3Y | Y | Y |
| Dystrophin (C-term) | Mouse IgG1 | Dy8/6C5 | Y | Y |
| Dystrophin (C-term) | Mouse IgG1 | MANDRA1 | Y | Y |
| Utrophin | Mouse IgG1 | DRP3/20C5 | Y | N |
| nNOS | Mouse IgG1 | RN5 | Y | N |
| Myotilin | Mouse IgG1 | RS034 | Y | N |
| Lamin A/C | Mouse IgG2b | 636 | Y* | N |
| Caveolin-3 | Mouse IgG1 | 26/Caveolin 3 | Y | Y |
| Calpain-3 | Mouse IgG2b | Calp3d/2C4 | N | Y |
| Calpain-3 | Mouse IgG2a | Calp3d/12A2 | N | Y |
| Dysferlin | Mouse IgG1 | Ham1/7B6 | Y | Y |
| Dysferlin | Mouse IgG2bκ | Ham3/17B2 | Y | N |
| α-Sarcoglycan | Mouse IgG1 | Ad1/20A6 | Y | Y |
| β-Sarcoglycan | Mouse IgG1 | b-sarc/5B1 | Y | N |
| γ-Sarcoglycan | Mouse IgG2bκ | 35DAG/21B5 | Y | Y |
| δ-Sarcoglycan | Mouse IgG2aκ | d-sarc3/12C1 | Y | N |
| Telethonin | Mouse IgG1 | G-11 | N | N |
| α-Dystroglycan | Mouse IgM | IIH6 | Y | Y |
| α-Dystroglycan | Mouse IgG1 | VIA4-1 | Y | Y |
| β-Dystroglycan | Mouse IgG2a | 43DAG1/8D5 | Y | Y |
| Laminin α2 80kDa | Mouse IgG1 | 5H2 | Y | Y |
| Laminin α2 300kDa | Mouse IgG1κ | Mer3/22B2 | Y | N |
| Laminin α2 300kDa | Rat IgG1 | 4H8 | Y | N |
| Laminin α5 | Mouse IgG2a | 4C7 | Y | N |
| Laminin β1 | Mouse IgG1κ | 4E10 | Y* | N |
| Laminin γ1 | Mouse IgG1κ | 2E8 | Y | N |
| Laminin γ1 | Mouse IgG2a | A5 | Y | N |
| Collagen VI | Mouse IgG1 | 3C4 | Y | N |
| Collagen VI | Rat IgG1κ | VI-26 | Y | N |
| Perlecan | Mouse IgG2aκ | A7L6 | Y | N |
| Emerin | Mouse IgG1 | 4G5 | Y | N |
| Desmin | Mouse IgG1 | D33 | Y | N |
| αB-Crystallin | Rabbit Polyclonal | G2JF | Y | N |
| VCP | Mouse IgG1 | 18/VCP | Y | N |
| PTRF-cavin | Mouse IgG1 | 4/PTRF | N | Y |
| MHC class I | Mouse IgG2a | W6-32 | Y | N |
| Neonatal Myosin | Mouse IgG1 | WB-MHCn | Y | N |
The list includes antibodies for the detection of primary or secondary defects and for the assessment of quality and preservation of samples.
*Immunoanalysis possible but not informative for diagnostic assessment.
IHC = immunohistochemistry; MHC = major histocompatibility complex; N = labelling not carried out routinely or antibody not tested/not suitable for test; nNOS = neuronal nitric oxide synthase; PTRF = polymerase I and transcript release factor; VCP = valosin-containing protein; WB = western blot; Y = labelling carried out routinely.