3-hydroxy-3-methylglutaryl-coenzyme A reductase
Lathosterol + NADPH + Oxygen ↔ 7-Dehydrocholesterol + NADP + 2 Water
7-Dehydrodesmosterol + NADPH ↔ 7-Dehydrocholesterol + NADP
Lathosterol → 5a-Cholest-8-en-3b-ol
7-Dehydrocholesterol + NADPH → Cholesterol + NADP
2 Acetyl-CoA ↔ Acetoacetyl-CoA + Coenzyme A
4,4-Dimethyl-5a-cholesta-8,24-dien-3-b-ol + 3 NADPH + 3 Oxygen → 4a-Methylzymosterol-4-carboxylic acid + 3 NADP + 4 Water
4a-Methylzymosterol-4-carboxylic acid + NADP → 3-Keto-4-methylzymosterol + Carbon dioxide + NADPH
NADPH + Zymosterol intermediate 2 → 5a-Cholest-8-en-3b-ol + NADP
5a-Cholesta-7,24-dien-3b-ol + NADPH ↔ Lathosterol + NADP
3-Keto-4-methylzymosterol + NADPH → 4a-Methylzymosterol + NADP
Zymosterol intermediate 2 → 5a-Cholesta-7,24-dien-3b-ol
3-Hydroxy-3-methylglutaryl-CoA + NADPH → Coenzyme A + Mevalonic acid + NADP
Cholesterol + Fatty acid → Cholesterol Ester + Water
Desmosterol + NADPH ↔ Cholesterol + NADP
Adenosine triphosphate + Mevalonic acid → Adenosine diphosphate + Mevalonic acid-5P
Adenosine triphosphate + Mevalonic acid-5P → (S)-5-Diphosphomevalonic acid + Adenosine diphosphate
(S)-5-Diphosphomevalonic acid + Adenosine triphosphate → Adenosine diphosphate + Carbon dioxide + Isopentenyl pyrophosphate + Phosphate
Lanosterol + 3 NADPH + 3 Oxygen → 4,4-Dimethylcholesta-8,14,24-trienol + Formic acid + 3 NADP + 4 Water
4,4-Dimethylcholesta-8,14,24-trienol + NADPH → 4,4-Dimethyl-5a-cholesta-8,24-dien-3-b-ol + NADP
Lanosterol + NADPH ↔ 24,25-Dihydrolanosterol + NADP
4,4-Dimethyl-5a-cholesta-8,24-dien-3-b-ol + NADPH → 4,4-Dimethyl-5a-cholesta-8-en-3b-ol + NADP
Isopentenyl pyrophosphate ↔ Dimethylallylpyrophosphate
2 Farnesyl pyrophosphate + NADPH → NADP + 2 Pyrophosphate + Squalene
NADPH + Oxygen + Squalene → (S)-2,3-Epoxysqualene + NADP + Water
Dimethylallylpyrophosphate + Isopentenyl pyrophosphate → Geranyl-PP + Pyrophosphate
Geranyl-PP + Isopentenyl pyrophosphate → Farnesyl pyrophosphate + Pyrophosphate
Acetoacetyl-CoA + Acetyl-CoA + Water → 3-Hydroxy-3-methylglutaryl-CoA + Coenzyme A
Chondrodysplasia Punctata II, X-Linked Dominant (CDPX2)
Lysosomal acid lipase/cholesteryl ester hydrolase
Valine, Leucine, and Isoleucine Degradation
7-Dehydrocholesterol → 8-Dehydrocholesterol
Hydroxymethylglutaryl-CoA synthase, mitochondrial
5a-Cholesta-7,24-dien-3b-ol → 7-Dehydrodesmosterol
4a-Methylzymosterol → Zymosterol intermediate 2