. 2021 Mar 31;22(7):3613. doi: 10.3390/ijms22073613

Table 1.

PWS mouse models and involved genes.

Gene(s) of Interest.	Name, Aliases	Phenotype	References
all (UPD Chr 7)	T(7;18)50H/+ (JAX001816, https://www.jax.org/strain/001816, accessed on 20 March 2021)	postnatal lethality (100%) growth retardation	[55] *
all (6.8 Mb deletion)	PWS^∆LMP2A Tg^PWS/AS(del) Del(7Herc2-Mkrn3)13FRdni/+	fetal growth retardation postnatal growth retardation neonatal lethality (100%) reduced movement irregular respiratory rate hypoglycemia pancreatic apoptosis insulin ↓, glucagon ↓ corticosterone ↑, ghrelin ↑	[40] * [56,57,58]
Frat3	Frat3^lacZ Peg12 ^tm1Brn	none	[30] *
Mkrn3	Mkrn3 ^m+/p−	lower weight from P45 earlier onset of puberty GnRH1 ↑	[54] *
Magel2	Magel2^m+/p− Magel2 KO C57BL/6-Magel2^tm1Stw/J (JAX009062, https://www.jax.org/strain/009062, accessed on 20 March 2021)	116HG expression ↓ postnatal lethality (~10%) reduced weight until P28 body fat ↑ lean mass ↓ bone mineral density ↓ impaired glucose homeostasis impaired cholesterol homeostasis insulin resistance leptin resistance dopamine ↓, serotonin ↓ adiponectin ↑ corticosterones ↑ oxytocin ↓ different feeding behavior less active anxiety impaired social behavior delayed onset of puberty progressive infertility	[53] * [59,60,61,62,63,64,65,66,67,68,69,70,71,72]
Magel2	Magel2 ^m+/p− Magel2 ^tm1.1Mus	postnatal lethality (~50%) weak suckling oxytocin ↓, orexin-A ↓ abnormal social behavior impaired learning ability	[52] * [73]
Magel2 (overexpression of truncated protein)	CAG-trMagel2	neonatal lethality (100%) small body size, poor suckling	[74] *
Necdin	Ndn ^m+/p− Ndn ^tm1Alb	none	[51] *
Necdin	Ndn^tm2Stw B6.129S1(Cg)-Ndn^tm2Stw/J (JAX009089, https://www.jax.org/strain/009089, accessed on 20 March 2021)	postnatal lethality (80–95% C57BL/6 and 25% FVB) respiratory distress	[48] * [75,76]
Necdin	Ndn^m+/p− B6.129S2-Ndn^tm1.1Mus	postnatal lethality (21–31%) respiratory distress oxytocin ↓ serotonin ↓	[50] * [77,78]
Necdin	Ndn ^m+/p− Ndn ^tm1Ky	respiratory abnormalities DRG neuron apoptosis ↑ pain sensitivity ↓ noradrenergic excitability ↓	[49] * [79]
Necdin	Ndn^m+/p− Necdin KO necdin ^m+/p−	unstable circadian rhythm	[80] *
Snurf/Snrpn Snord116 IPW Snord115 Ube3a	Snrpn-Ube3a deletion Del(7Ube3a-Snrpn)1Alb	neonatal lethality (80%) growth retardation hypotonia	[41] *
all (IC deletion)	PWS-IC^∆35kb Snrpn^tm2Cbr ∆PWS-IC PWS-IC^del PWS-IC^del35kb B6.129-Snrpn^tm2Cbr/J (JAX012443, https://www.jax.org/strain/012443, accessed on 20 March 2021)	neonatal lethality (40–90% depending on background) growth retardation hypotonia decreased locomotive ability abnormal behavior ghrelin ↑ increased food consumption food-seeking behavior ↑	[45] * [81,82,83,84,85,86]
all (IC deletion)	PWS-IC ^{m+/p del4.8kb} PWS-IC ^∆4.8 Snrpn ^tm2Alb	neonatal lethality (40%) growth retardation	[46] *
all, except Snrpn, Snord64, 116, 115 (IC deletion)	PWS-IC ^Hs Snrpn ^tm1Kaj	neonatal lethality (47% C57BL/6J and 16% 129S1/Sv) growth retardation feeding difficulties	[87] *
all (IC deletion)	PWS-IC^m+/p∆6kb PWS-IC^∆6kb B6.129S1-Snrpn^tm2.1Kaj/J (JAX018395, https://www.jax.org/strain/018395, accessed on 20 March 2021)	neonatal lethality (100%) growth retardation feeding difficulties	[47] *
Snord116/IPW	PWScr^m+/p− Del(7Ipw-Snord116)1Jbro (distributed by TRAM Münster)	neonatal lethality (15%) growth retardation pOx ↑, Peg3 ↑ decreased gray-matter volume altered sleep profile altered body temperature	[42] * [88,89]
Snord116/IPW	Snord116del Snord116^tm1Uta Snord116^+/−P B6(Cg)-Snord116^tm1.1Uta/J (JAX008149, https://www.jax.org/strain/008149, accessed on 20 March 2021)	growth retardation Igf1 ↓ ghrelin ↓ impaired pancreatic development altered diurnal methylation increased anxiety altered respiratory exchange rate resistant to obesity	[43] * [90,91,92,93,94]
Snord116/IPW (homozygous)	Snord116 ^m−/p− Snord116 ^−/−	growth retardation fat mass ↓ increased food consumption altered diurnal activity profile resistant to obesity altered hypothalamic signaling	[95] *
Snord116/IPW (only Npy⁺ Neurons)	Snord116^lox/lox/NPY^cre/+ (JAX008118, https://www.jax.org/strain/008118, accessed on 20 March 2021)	growth retardation fat mass ↓ increased food consumption altered diurnal activity profile altered hypothalamic signaling	[95] *
Snord116/IPW (adult-onset)	Snord116 deletion	reduced food consumption insulin resistance	[96] *
Snord116/IPW (adult-onset)	AAV-Snord116del^m^+/p− Snord116^flAAV-Cre	increased food consumption bodyweight ↑ bodyfat percentage ↑	[91] *
Snord115	Snord115-deficient	brown adipose tissue ↑ modest alterations in 5-Htr2cr mRNA A-to-I editing	[44] *
Snord116 (single copy transgene)		no effect on phenotype	[43] *
Snord116 (transgene 2 mouse, 1 rat copies)	PWScr^m+/p−TgSnord116	no effect on phenotype	[97] *
Snord116 (transgene 27 copies)		no effect on phenotype	[98] *
all (biallelic IC deletion)	PWS-IC^{m∆4.8kB/p∆4.8kB} PWS-IC^{m∆4.8kB/p∆S-U}	rescue of postnatal lethality rescue of growth retardation	[99]
Snord116/IPW (maternal IC deletion)	PWScr^{m5′LoxP/p−} (distributed by TRAM Münster)	Rescue of growth retardation in adult mice alterations in 5-Htr2cr mRNA A-to-I editing in the choroid plexus.	[97] *
Snord116 (AAV-mediated)	Snord116del^m−/p−AAV-Snord116	energy expenditure ↑ rate of weight gain ↓	[100] *

Original publications are marked by *, up- and downregulation of physiological parameters is represented by arrows (↑ and ↓).