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Lmx1b is member of Lmx subgroup of LIM-HD protiens, together with Lmx1a. Lmx1b is widely expressed in the developing mouse embryo, and it has a role in limb, eye, kidney, brain and skull development. In the CNS, Lmx1b is required for formation of the isthmus, an important signaling center at the midbrain/hindbrain junction, where it maintains Wnt1 and Fgf8 expression. Lmx1b is also required for hindbrain serotonergic and midbrain dopaminergic neuronal development. It also controls roof plate formation and differentiation and migration of a subset of dorsal dI3 inerneurons in the spinal cord. In the developing limbs, Lmx1b coordinates the dorsoventral trajectories of incoming motor neurons. Heterozygous mutations in human LMX1B cause nail patella syndrome, which is modeled by Lmx1b null mice which have limb and kidney abnormalities in addition to the CNS phenotypes outlined above.