Phenotypes associated with this allele

• Allele Symbol: Tlx2^{tm1.1(cre)Rpk}
• Allele Name: targeted mutation 1.1, Raj P Kapur
• Allele ID: MGI:3846678
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Tlx2^tm1.1(cre)Rpk/Tlx2^tm1.1(cre)Rpk	involves: 129S4/SvJae * C57BL/6	MGI:3847080
cn2	Prkar1a^tm2Gsm/Prkar1a^tm2Gsm Tlx2^tm1.1(cre)Rpk/Tlx2^+	involves: 129S4/SvJae * 129X1/SvJ * C57BL/6 * C57BL/6N * DBA/2	MGI:3847081

Genotype
MGI:3847080

hm1

• Allelic Composition: Tlx2^{tm1.1(cre)Rpk}/Tlx2^{tm1.1(cre)Rpk}
• Genetic Background: involves: 129S4/SvJae * C57BL/6

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

mortality/aging

premature death ( J:148830 )

• mice die shortly after weaning with cecal and proximal colonic distention

digestive/alimentary system

distended cecum ( J:148830 )

• at P15

megacolon ( J:148830 )

• at P15

abnormal small intestine morphology ( J:148830 )

• at P15, the distal small intestine is dilated

growth/size/body

distended abdomen ( J:148830 )

• at P15, 71% of mice develop lethally distended abdomens due to massive dilation of their cecum and distal small intestine unlike wild-type mice

• however, myenteric ganglion cell densities are normal

Genotype
MGI:3847081

cn2

• Allelic Composition: Prkar1a^tm2Gsm/Prkar1a^tm2Gsm; Tlx2^{tm1.1(cre)Rpk}/Tlx2⁺
• Genetic Background: involves: 129S4/SvJae * 129X1/SvJ * C57BL/6 * C57BL/6N * DBA/2

digestive/alimentary system

digestive/alimentary phenotype ( J:148464 )

N • despite disruptions in peristalsis, mice exhibit normal myenteric neuron density and enteric neuron distribution

distended duodenum ( J:148464 )

• distended

intestinal hypoperistalsis ( J:148464 )

• inter-migrating motility complex (MCC) intervals increased with age

• regular initiation of MMCs within the proximal colon and robust distal propagation are disrupted

• mice exhibit irregular gastrointestinal tract motor activity and severely impaired peristalsis compared with wild-type mice

abnormal small intestinal transit time ( J:148464 )

• slowed

intestinal obstruction ( J:148464 )

• mice exhibit a similar pseudo-obstruction phenotype observed in Prkar1a^tm1Rpk/Prkar1a^tm1Rpk Tg(Plp1-cre)1Bzal mice

craniofacial

craniofacial phenotype ( J:148464 )

N • unlike Prkar1a^tm1Rpk/Prkar1a^tm1Rpk Tg(Plp1-cre)1Bzal mice, no craniofacial abnormalities are observed

muscle

intestinal hypoperistalsis ( J:148464 )

• regular initiation of MMCs within the proximal colon and robust distal propagation are disrupted

• inter-migrating motility complex (MCC) intervals increased with age

• mice exhibit irregular gastrointestinal tract motor activity and severely impaired peristalsis compared with wild-type mice