| CDBP00012 | Pyruvate dehydrogenase E1 component subunit beta, mitochondrial | P11177 | PDHB | 3p21.1-p14.2 | Enzyme | Not Available |
| CDBP00016 | Malonyl-CoA decarboxylase, mitochondrial | O95822 | MLYCD | 16q24 | Enzyme | Not Available |
| CDBP00017 | 5-aminolevulinate synthase, erythroid-specific, mitochondrial | P22557 | ALAS2 | Xp11.21 | Enzyme | Not Available |
| CDBP00022 | 5-aminolevulinate synthase, nonspecific, mitochondrial | P13196 | ALAS1 | 3p21.1 | Enzyme | Not Available |
| CDBP00033 | Fatty acid synthase | P49327 | FASN | 17q25 | Enzyme | Not Available |
| CDBP00044 | Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial | P08559 | PDHA1 | Xp22.1 | Enzyme | Not Available |
| CDBP00048 | Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial | P29803 | PDHA2 | 4q22-q23 | Enzyme | Not Available |
| CDBP00049 | Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial | Q02252 | ALDH6A1 | 14q24.3 | Enzyme | Not Available |
| CDBP00052 | Dihydrolipoyl dehydrogenase, mitochondrial | P09622 | DLD | 7q31-q32 | Enzyme | Not Available |
| CDBP00053 | Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial | P10515 | DLAT | 11q23.1 | Enzyme | Not Available |
| CDBP00084 | Tyrosinase | P14679 | TYR | 11q14-q21 | Enzyme | Not Available |
| CDBP00197 | Glutaryl-CoA dehydrogenase, mitochondrial | Q92947 | GCDH | 19p13.2 | Enzyme | Not Available |
| CDBP00221 | Lysosomal acid lipase/cholesteryl ester hydrolase | P38571 | LIPA | 10q23.2-q23.3 | Enzyme | Not Available |
| CDBP00244 | Uroporphyrinogen decarboxylase | P06132 | UROD | 1p34 | Enzyme | Not Available |
| CDBP00245 | Coproporphyrinogen-III oxidase, mitochondrial | P36551 | CPOX | 3q12 | Enzyme | Not Available |
| CDBP00255 | 2-amino-3-carboxymuconate-6-semialdehyde decarboxylase | Q8TDX5 | ACMSD | 2q21.3 | Enzyme | Not Available |
| CDBP00257 | Methylsterol monooxygenase 1 | Q15800 | MSMO1 | 4q32-q34 | Enzyme | Not Available |
| CDBP00258 | Sterol-4-alpha-carboxylate 3-dehydrogenase, decarboxylating | Q15738 | NSDHL | Xq28 | Enzyme | Not Available |
| CDBP00264 | Phytanoyl-CoA dioxygenase, peroxisomal | O14832 | PHYH | 10p13 | Enzyme | Not Available |
| CDBP00272 | Aromatic-L-amino-acid decarboxylase | P20711 | DDC | 7p12.2 | Enzyme | Not Available |
| CDBP00275 | Peroxiredoxin-6 | P30041 | PRDX6 | 1q25.1 | Enzyme | Not Available |
| CDBP00293 | 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial | P21953 | BCKDHB | 6q14.1 | Enzyme | Not Available |
| CDBP00294 | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial | P12694 | BCKDHA | 19q13.1-q13.2 | Enzyme | Not Available |
| CDBP00296 | Prolyl 4-hydroxylase subunit alpha-2 | O15460 | P4HA2 | 5q31 | Enzyme | Not Available |
| CDBP00298 | Prolyl 4-hydroxylase subunit alpha-1 | P13674 | P4HA1 | 10q21.3-q23.1 | Enzyme | Not Available |