Phenotypes Associated with This Genotype

Genotype
MGI:5440759

• Allelic Composition: Lrp4^tm1.1Line/Lrp4^tm1.1Line; Tg(ACTA1-cre)79Jme/0
• Genetic Background: involves: 129S/SvEv * C57BL/6 * C57BL/6J * SJL

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

postnatal lethality, incomplete penetrance ( J:188352 )

• most mice do not die until P15

• few mice survive beyond the postnatal period

nervous system

abnormal motor neuron morphology ( J:188352 )

• increased number and length of secondary or intramuscular branches with tertiary and quaternary branches

• motor axons fail to terminate at AChR clusters

• however, primary nerve branches are normal

abnormal neuromuscular synapse morphology ( J:188352 )

• abnormal AChR clusters in the diaphragm with wider area in the middle of muscle fibers, increased endplate band-width (at P0 and P10), elongated morphology, reduced average cluster size and increased cluster number

• ectopic AChR clusters

abnormal endplate potential ( J:188352 )

• decreased amplitudes of endplate potentials

abnormal miniature endplate potential ( J:188352 )

• smaller than in control mice