Table 1.
Mouse models of processing enzyme insufficiency
| Enzyme | Mouse modela | Description | Obesityb | Relative αMSH levels | Reference |
|---|---|---|---|---|---|
| CPE | |||||
| Cpe fat (S244P) | Global point mutant | Yes | Reduced > 10-fold (hypothalamus and pituitary) | (38) | |
| Cpe knockout | Global | Yes | Reduced > 10-fold (hypothalamus and pituitary) | (39) | |
| CpeFlox::PomcCre knockout | Floxed; conditional | No | Reduced > 10-fold (hypothalamus and pituitary) | (40) | |
| PC1/3 | |||||
| Pcsk1 knockout | Global | Noc | Normal (pituitary) | (41) | |
| Pcsk1Flox::PomcCre knockout | Floxed; conditional | No | Normal (hypothalamus) | (42) | |
| Pcsk1-N222D | Point mutant | Yes | Reduced by half (hypothalamus) | (43) | |
| Pcsk1-N221D | Point mutant | No | Normal (hypothalamus) | (44) | |
| Pcsk1-V96L;exon3 del | Exon3 splicing mutant and point mutant | Yes | Not measured | (45) | |
| PC2 | Pcsk2 knockout | Global | No | Reduced > 10-fold (below detection limit in pituitary and hypothalamus) | (46-49) |
| PAM | Pam knockout, heterozygous | Global | No | Normal (hypothalamus) | (50) |
Abbreviations: αMSH, α-melanocyte-stimulating hormone; CPE, carboxypeptidase E; PAM, peptidyl-α-amidating monooxygenase; PC1/3, prohormone convertase 1.
a Homozygous unless indicated.
b Obesity on normal chow diet.
c Depends on mouse strain.