| CDBP05121 | USP21 | 1q22 | Ubiquitin carboxyl-terminal hydrolase 21 | Enzyme | 1 |
| CDBP05139 | USP20 | 9q34.11 | Ubiquitin carboxyl-terminal hydrolase 20 | Enzyme | 1 |
| CDBP05130 | USP2 | 11q23.3 | Ubiquitin carboxyl-terminal hydrolase 2 | Enzyme | 1 |
| CDBP04685 | USP17A | | Ubiquitin carboxyl-terminal hydrolase 17 | Enzyme | 0 |
| CDBP04613 | USP16 | 21q22.11 | Ubiquitin carboxyl-terminal hydrolase 16 | Enzyme | 1 |
| CDBP04637 | USP15 | 12q14 | Ubiquitin carboxyl-terminal hydrolase 15 | Enzyme | 1 |
| CDBP04633 | USP13 | 3q26.2-q26.3 | Ubiquitin carboxyl-terminal hydrolase 13 | Enzyme | 1 |
| CDBP05110 | USP11 | | Ubiquitin carboxyl-terminal hydrolase 11 | Enzyme | 1 |
| CDBP04635 | USP10 | 16q24.1 | Ubiquitin carboxyl-terminal hydrolase 10 | Enzyme | 1 |
| CDBP04625 | USP1 | 1p31.3 | Ubiquitin carboxyl-terminal hydrolase 1 | Enzyme | 1 |
| CDBP00350 | UROS | 10q25.2-q26.3 | Uroporphyrinogen-III synthase | Enzyme | 2 |
| CDBP00244 | UROD | 1p34 | Uroporphyrinogen decarboxylase | Enzyme | 3 |
| CDBP00857 | UROC1 | 3q21.3 | Urocanate hydratase | Enzyme | 4 |
| CDBP01868 | UQCRQ | 5q31.1 | Cytochrome b-c1 complex subunit 8 | Enzyme | 1 |
| CDBP00930 | UQCRH | 1p34.1 | Cytochrome b-c1 complex subunit 6, mitochondrial | Transporter | 2 |
| CDBP00928 | UQCRFS1 | 19q12 | Cytochrome b-c1 complex subunit Rieske, mitochondrial | Enzyme | 3 |
| CDBP00923 | UQCRC2 | 16p12 | Cytochrome b-c1 complex subunit 2, mitochondrial | Metal Binding | 1 |
| CDBP00925 | UQCRC1 | 3p21.3 | Cytochrome b-c1 complex subunit 1, mitochondrial | Metal Binding | 1 |
| CDBP00926 | UQCRB | 8q22 | Cytochrome b-c1 complex subunit 7 | Enzyme | 1 |
| CDBP02248 | UQCR11 | 19p13.3 | Cytochrome b-c1 complex subunit 10 | Transporter | 2 |
| CDBP00924 | UQCR10 | 22cen-q12.3 | Cytochrome b-c1 complex subunit 9 | Transporter | 2 |
| CDBP04699 | UPRT | | Uracil phosphoribosyltransferase homolog | Enzyme | 4 |
| CDBP02477 | UPP2 | 2q24.1 | Uridine phosphorylase 2 | Enzyme | 2 |
| CDBP02476 | UPP1 | 7p12.3 | Uridine phosphorylase 1 | Enzyme | 2 |
| CDBP00516 | UPB1 | 22q11.2 | Beta-ureidopropionase | Enzyme | 5 |