Homozygous mutant mice are born with no overt morphological defects but display severe abdominal distension and nearly all die within 24 hours of birth. Several defects in motor neuron differentiation are observed at thoracic levels of the spinal cord. Many HB9+ (MNX1+) motor neurons are found in ectopic locations, either in intermediate areas of the spinal cord or within the ventral roots. In addition, the tight columnar organization of median motor column (MMC) neurons evident at thoracic levels of wild-type embryos are disrupted in mutants, with an intermingling of neurons in the medial and lateral divisions of the MMC, defined by their LHX3 (medial MMC motor neuron)/HB9 (ectopic motor neuron) expression status. 

Homozygous embryos lack ISL2 protein expression. At E9.5, ISL1 expression is indistinguishable in mutant and wild-type embryos, but by E10.5 ISL1 expression is absent from a small group of medially positioned motor neurons in Isl2 mutants. By E11.5, the spatial segregation between HB9+/ISL1+ and HB9+/ISL1- cells becomes more distinct as HB9+ /ISL1- neurons begin to migrate dorsally. The total number of motor neurons at brachial, lumbar, and thoracic levels is similar in wild-type and Isl2 mutants. This strain may be useful to studies of neurodevelopment.

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Homozygous Isl2 (insulin related protein 2 (islet 2)) targeted mutant mice are born with no overt morphological defects but display severe abdominal distension and nearly all die within 24 hours of birth. Several defects in motor neuron differentiation are observed at thoracic levels of the spinal cord. Many HB9+ (MNX1+) motor neurons are found in ectopic locations, either in intermediate areas of the spinal cord or within the ventral roots. In addition, the tight columnar organization of median motor column (MMC) neurons evident at thoracic levels of wild-type embryos are disrupted, with an intermingling of neurons in the medial and lateral divisions of the MMC, defined by their LHX3 (medial MMC motor neuron)/HB9 (ectopic motor neuron) expression status. This strain may be useful for studies of neurodevelopment.

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