Mouse Genome Informatics
ht
    Ndntm2Stw/Ndn+
involves: 129S1/Sv * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• mice inheriting the mutant allele paternally die a few hours after birth

respiratory system
• the respiratory motor discharge pattern produced by cervical ventral roots, hypoglossal roots, cranial motoneuron pools and within neurons located in the putative respiratory rhythm-generating center, the pre-Botzinger complex, is very irregular in mice inheriting the mutant allele from the father, with prominent bouts of depression of respiratory rhythmogenesis
• mice inheriting the mutant allele paternally gasp for air when born and turn cyanotic
• mice inheriting the mutant allele paternally exhibit hypoventilation resulting from a defective central respiratory drive

cellular

homeostasis/metabolism
• in mutants with the paternally inherited allele
• in mutants with the paternally inherited allele

nervous system
• pups with abnormal breathing fail to generate rhythmic motor bursts from cervical or hypoglossal nerve roots in vitro or generate a severely irregular rhythmic motor output
• he respiratory motor discharge pattern produced by cervical ventral roots, hypoglossal roots, cranial motoneuron pools and within neurons located in the putative respiratory rhythm-generating center, the pre-Botzinger complex, is very irregular in mice inheriting the mutant allele from the father, with prominent bouts of depression of respiratory rhythmogenesis

Mouse Models of Human Disease
OMIM IDRef(s)
Prader-Willi Syndrome; PWS 176270 J:82266