| First Author | Hadano S | Year | 2010 |
| Journal | Neurosci Res | Volume | 68 |
| Issue | 2 | Pages | 131-6 |
| PubMed ID | 20558214 | Mgi Jnum | J:165755 |
| Mgi Id | MGI:4838385 | Doi | 10.1016/j.neures.2010.06.004 |
| Citation | Hadano S, et al. (2010) Genetic background and gender effects on gross phenotypes in congenic lines of ALS2/alsin-deficient mice. Neurosci Res 68(2):131-6 |
| abstractText | Loss-of-function mutations in human ALS2 account for several juvenile recessive motor neuron diseases (MNDs). To understand the molecular basis underlying motor dysfunction in ALS2-linked MNDs, several lines of Als2(-/-) mice with a mixed genetic background were thus far generated, and their phenotypes were thoroughly characterized. However, several phenotypic discrepancies among different Als2-deficient lines became evident. To investigate whether genetic backgrounds are associated with such discrepancies, we here generated congenic lines of Als2(-/-) mice on two different genetic backgrounds; C57BL/6 (B6) and FVB/N (FVB), and investigated their gross phenotypes. Both B6 and FVB congenic lines were viable and fertile with no evidences for obvious abnormalities. There were no differences in growth curves between wild-type and Als2(-/-) mice on each genetic background. Remarkably, Als2(-/-) mice on a FVB, but not a B6, background exhibited a shorter life span than wild-type litters. Further, B6 female, but not male, Als2(-/-) mice showed a significantly lower spontaneous rearing activity than wild-type litters. These genetic background- and/or gender-specific findings suggest the presence of modifiers for life span and motor activities in Als2(-/-) mice. These congenic mice should provide a useful means to understand the molecular and genetic basis for variable expression of pathological phenotypes in MNDs. |
