Table 1

Summary of results for Df(h22q11)/+ and other 22q11.2DS mouse models

ModelDf(h22q11)/+Df(16)A+/−LgDelDf1/+
DeletionDgcr2-HiraDgcr2-HiraDgcr2-HiraDgcr14-Ufd11Znf74-Ctp
StrainC57/Bl6NC57/Bl6JC57/Bl6NMixed C57/Bl6C-/C-; 129S5/SvEvBrd129SvEvTac or mixed 129SvEvTac
Phenotype; measure
 PPI
  Pre-puberty
  Post-puberty (18) (16) (17) (15)
  Clozapine or haloperidol challengesx
 Acoustic startle
  Pre-puberty
  Post-pubertyx( (17)) (16) (17)x( (15))
  Clozapine or haloperidol challengesx
Stimulant LMA
 PCP
  Pre-pubertyx
  Post-puberty
  Ketamine (post-puberty)
Electrophysiology
 Prefrontal spike frequency
  Baselinex
  Gabazine challengex
  Cortical low frequency oscillation
  Baselinex
  PCP challengex
  LDAEP
  ABRx (19)
Biochemistry
 hPLC (PFC and DStr)
  DOPAC
  DA, 5-HT, NA, 5-HIAA, HVAx
  DStr microdialysis
  Baseline DA, DOPAC, Glu, HVAx
  Veratridine, nomifensine, PCP challengex
Western analysis (PFC, DStr, Hipp)
 Synaptic (PSD-95, SYP, Syn1, Drebrin, geophysin)x
 Cell type (NeuN, GFAP)x
 GABA (GABAAα1, KCC2, VGAT, GAD65/67)x
 Glutamate (GluR2, NR2A, NR2B, VGluT1)x
 DStr GluR1
  • 5-HIAA = 5-hydroxyindoleacetic acid; 5-HT = 5-hydroxytryptamine; ABR = auditory induced brainstem response; DA = dopamine; DOPAC = dihydroxyphenylacetic acid; DStr = dorsal striatum; GABA = γ-aminobutyric acid; Hipp = hippocampus; HVA = homovanillic acid; LDAEP = loudness dependence auditory evoked potentials; LMA = locomotor activity; NA = noradrenaline; PCP = phencyclidine; PFC = prefrontal cortex; PPI = prepulse inhibition; ↓ = decreased; ↑ = increased; x = no effect; () = did not replicate.