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The Abelson-helper integration site 1 (AHI1) gene encodes for a ciliary transition zone localizing protein that when mutated causes the human ciliopathy, Joubert syndrome. We prepared and examined neuronal cultures derived from male and female embryonic Ahi1^+/+ and Ahi1^–/– mice (littermates) and found that the distribution of ciliary melanin-concentrating hormone receptor-1 (MchR1) was significantly reduced in Ahi1^–/– neurons; however, the total and surface expression of MchR1 on Ahi1^–/– neurons was similar to controls (Ahi1^+/+). This indicates that a pathway for MchR1 trafficking to the surface plasma membrane is intact, but the process of targeting MchR1 into cilia is impaired in Ahi1-deficient mouse neurons, indicating a role for Ahi1 in localizing MchR1 to the cilium. Mouse Ahi1^–/– neurons that fail to accumulate MchR1 in the ciliary membrane have significant decreases in two downstream MchR1 …