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The human  CLCA4  (chloride channel regulator, calcium-activated) modulates the intestinal phenotype of cystic fibrosis (CF) patients via an as yet unknown pathway. With the generation of new porcine CF models, species-specific differences between human modifiers of CF and their porcine orthologs are considered critical for the translation of experimental data. Specifically, the porcine ortholog to the human CF modulator gene  CLCA4  has recently been shown to be duplicated into two separate genes,  CLCA4a  and  CLCA4b . Here, we characterize the duplication product,  CLCA4b , in terms of its genomic structure, tissue and cellular expression patterns as well as its  in vitro  electrophysiological properties. The  CLCA4b  gene is a pig-specific duplication product of the  CLCA4  ancestor and its protein is exclusively expressed in small and large intestinal crypt epithelial cells, a niche specifically occupied by no other porcine  CLCA  family member. Surprisingly, a unique deleterious mutation of the  CLCA4b  gene is spread among modern and ancient breeds in the pig population, but this mutation did not result in an apparent phenotype in homozygously affected animals. Electrophysiologically, neither the products of the wild type nor of the mutated  CLCA4b  genes were able to evoke a calcium-activated anion conductance, a consensus feature of other  CLCA  proteins. The apparently pig-specific duplication of the  CLCA4  gene with unique expression of the  CLCA4b  protein variant in intestinal crypt epithelial cells where the porcine CFTR is also present raises the question of whether it may modulate the porcine CF phenotype. Moreover, the naturally occurring null variant of  CLCA4b  will be valuable for the understanding of  CLCA  protein function and their relevance in modulating the CF phenotype.

Naturally Occurring Deletion Mutants of the Pig-Specific, Intestinal Crypt Epithelial Cell Protein CLCA4b without Apparent Phenotype