Caenorhabditis elegans


CELE_R13H4.1, nph-4, R13H4.1
nphp-4 encodes an ortholog of human NPHP4 (OMIM:607215, mutated in juvenile nephronophthisis and Senior-Loken syndrome-4) that is required for normal chemotaxis, lifespan, and male mating behavior; NPHP-4 is expressed in diverse neurons (amphid and phasmid sensory, URX, labial, male lumbar and cloacal ganglia, and male-specific CEM, HOB and RnB); within neurons, NPHP-4 is a ciliary protein, localized to the transistion zone at cilial bases rather than ciliary axonemes, and absent from somata, axons, or dendrites; NPHP-4 colocalizes with NPHP-1 and PKD-2 in male-specific sensory cilia, and is required for the normal ciliary localization of NPHP-1; NPHP-4 expression requires DAF-19, and mutating an X-box in nphp-4's promoter abolishes nphp-4 expression; NPHP-4 is required for NPHP-1's localization to transistion zones; morphologically, nphp-4 mutant cilia are normal, indicating a function for NPHP-4 in signal transduction.
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