Table 1.
Baseline characterization of Ae1+/R607H and Ae1R607H/R607H mice
Characteristics | Units | Ae1+/+ | n | Ae1+/R607H | n | Ae1R607H/R607H | n |
---|---|---|---|---|---|---|---|
General | |||||||
BW | g | 24.45±0.39 | 6 | 24.11±0.31 | 10 | 23.76±0.48 | 8 |
Food intake | g/gBW per d | 0.17±0.01 | 6 | 0.17±0.01 | 10 | 0.17±0.01 | 8 |
Water intake | ml/gBW per d | 0.18±0.02 | 6 | 0.18±0.01 | 10 | 0.18±0.01 | 8 |
Blood | |||||||
Hct | % | 45±0.65 | 13 | 45±0.28 | 12 | 47±0.80 | 9 |
Hb | g/dl | 15.1±0.16 | 9 | 15.08±0.16 | 13 | 15.3±0.36 | 15 |
Na+ | mM | 146±0.36 | 13 | 147±0.46 | 12 | 147±0.47 | 9 |
Cl- | mM | 111±0.74 | 13 | 115±0.77* | 13 | 118±1.44**** | 13 |
K+ | mM | 4.56±0.10 | 7 | 4.75±0.07 | 7 | 4.45±0.08 | 5 |
Ca2+ | mM | 1.25±0.01 | 13 | 1.26±0.01 | 13 | 1.25±0.01 | 12 |
pH | 7.32±0.02 | 13 | 7.32±0.01 | 13 | 7.29±0.02 | 12 | |
pCO2 | mmHg | 43±1.19 | 12 | 46±1.16 | 12 | 43±0.92 | 11 |
HCO3– | mM | 22±0.55 | 13 | 22±0.44 | 12 | 21±0.70 | 12 |
Urine | |||||||
Urine output | ml/gBW per d | 0.075±0.008 | 6 | 0.069±0.005 | 10 | 0.065±0.004 | 8 |
Urine Osm | mOsm/kgH2O | 3178±383 | 6 | 2891±246 | 10 | 2670±108 | 8 |
Urine pH | 6.45±0.04 | 6 | 6.95±0.05**** | 10 | 7.09±0.05**** | 8 | |
Na+/Creat | mmol/mmol | 17±1.19 | 6 | 20±0.68 | 10 | 21±0.93 | 8 |
K+/Creat | mmol/mmol | 57±11.69 | 6 | 63±6.41 | 10 | 64±5.99 | 8 |
Ca2+/Creat | mmol/mmol | 0.17±0.02 | 6 | 0.15±0.01 | 10 | 0.10±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.