Table 2.

Characteristics of the human LCs used in the study

LCKidney DiseaseIsotypeV (L) DomainMutations of InterestaCrystal FormationbpI (V Domain)Charge (pH 7/pH 5)Aggregation Score (V Domain)cReference
CHRFSκVκ1–39A30; I94Yes8.451.9/3.2486.691,6
DURFSκVκ1–5V31Yes5.17−1.1/0.4897.65
CHmκVκ1–39A30→SNo8.451.9/3.2229.126
DEAL amyloidosisκVκ1–33F52No5.36−1.1/1.0332.77
ROCast nephropathyκVκ2–28A51;Y52No7.090.1/3.0595.71
  • V(L), variable domain; pl, isoelectric point; DU, RFS-kLC; —, not causing kidney disease; AL, amyloid light chain; CDR, complementary determing region; TANGO, algorithm used to predict the aggregating regions in the V(L) domain.

  • a Polar to hydrophobic residue substitution in solvent-exposed loops (CDR).

  • b Crystal formation was documented by electron microscopy study of the kidney biopsy.

  • c Aggregation score obtained using the TANGO algorithm.