Extrarenal Signs of Proximal Renal Tubular Acidosis Persist in Nonacidemic Nbce1b/c-Null Mice.
Emily E SalernoSangita P PatelAniko MarshallJordan MarshallThamer AlsufayanCheikh S Alassane MballoBianca N QuadeMark D ParkerPublished in: Journal of the American Society of Nephrology : JASN (2019)
The correction of pRTA-related acidemia should not be considered a panacea for all signs of pRTA. The phenotype of Nbce1b/c-null mice highlights the physiologic importance of NBCe1 variants expressed beyond the proximal tubular epithelia and potential limitations of pH correction by alkali therapy in pRTA. It also suggests a novel genetic locus for corneal dystrophy and enamel hypomineralization without acidemia.