Table 1.

Baseline characterization of Ae1+/R607H and Ae1R607H/R607H mice

CharacteristicsUnitsAe1+/+nAe1+/R607HnAe1R607H/R607Hn
General
 BWg24.45±0.39624.11±0.311023.76±0.488
 Food intakeg/gBW per d0.17±0.0160.17±0.01100.17±0.018
 Water intakeml/gBW per d0.18±0.0260.18±0.01100.18±0.018
Blood
 Hct%45±0.651345±0.281247±0.809
 Hbg/dl15.1±0.16915.08±0.161315.3±0.3615
 Na+mM146±0.3613147±0.4612147±0.479
 Cl-mM111±0.7413115±0.77*13118±1.44****13
 K+mM4.56±0.1074.75±0.0774.45±0.085
 Ca2+mM1.25±0.01131.26±0.01131.25±0.0112
 pH7.32±0.02137.32±0.01137.29±0.0212
 pCO2mmHg43±1.191246±1.161243±0.9211
 HCO3mM22±0.551322±0.441221±0.7012
Urine
 Urine outputml/gBW per d0.075±0.00860.069±0.005100.065±0.0048
 Urine OsmmOsm/kgH2O3178±38362891±246102670±1088
 Urine pH6.45±0.0466.95±0.05****107.09±0.05****8
 Na+/Creatmmol/mmol17±1.19620±0.681021±0.938
 K+/Creatmmol/mmol57±11.69663±6.411064±5.998
 Ca2+/Creatmmol/mmol0.17±0.0260.15±0.01100.10±0.01*8
  • In the basal state, Ae1+/R607H and Ae1R607H/R607H mice have no detectable alterations in acid-base parameters, revealing that, as in humans, R607H mutation causes incomplete dRTA with a higher urinary pH. The increase in blood chloride levels is consistent with the presence of a mild hyperchloremic metabolic acidosis. Values are means±SEM. *P<0.05; ****P<0.0001 versus Ae1+/+ (age 3–5 months, one-way ANOVA). BW, Body weight; gBW, Gram body weight; Hct, Hematocrit; Hb, Hemoglobin; Osm, Osmolarity; mOsm, Milliosmoles; Creat, Creatinine.