Congenital Lipoid Adrenal Hyperplasia (CLAH) or Lipoid CAH

Metabolites

11-Deoxycortisol

Formula: C21H30O4 (346.214398)

CAS ID: 152-58-9

18-Hydroxycorticosterone

Formula: C21H30O5 (362.209313)

CAS ID: 561-65-9

21-Deoxycortisol

Formula: C21H30O4 (346.214398)

CAS ID: 641-77-0

4-Methylpentanal

Formula: C6H12O (100.0888102)

CAS ID: 1119-16-0

Cholesterol

Formula: C27H46O (386.3548466)

CAS ID: 57-88-5

Corticosterone

Formula: C21H30O4 (346.214398)

CAS ID: 50-22-6

Cortisol

Formula: C21H30O5 (362.209313)

CAS ID: 50-23-7

Cortisone

Formula: C21H28O5 (360.1936638)

CAS ID: 53-06-5

Dihydrocortisol

Formula: C21H32O5 (364.2249622)

CAS ID: 1482-50-4

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

NAD+

Formula: C21H28N7O14P2 (664.1169428000001)

CAS ID: 53-84-9

NADH

Formula: C21H29N7O14P2 (665.1247674)

CAS ID: 58-68-4

NADP+

Formula: C21H29N7O17P3 (744.0832754)

CAS ID: 53-59-8

Oxygen

Formula: O2 (31.98983)

CAS ID: 7782-44-7

Pregnanolone

Formula: C21H34O2 (318.2558664)

CAS ID: 128-20-1

Progesterone

Formula: C21H30O2 (314.224568)

CAS ID: 57-83-0

Tetrahydrocortisone

Formula: C21H32O5 (364.2249622)

CAS ID: 53-05-4



Enzyme

EC Number name full name note
1.1.1.-
1.1.1.145 3beta-hydroxy-DELTA5-steroid dehydrogenase 3beta-hydroxy-DELTA5-steroid:NAD+ 3-oxidoreductase
1.1.1.146 11beta-hydroxysteroid dehydrogenase 11beta-hydroxysteroid:NADP+ 11-oxidoreductase
1.1.1.201 7beta-hydroxysteroid dehydrogenase (NADP+) 7beta-hydroxysteroid:NADP+ 7-oxidoreductase
1.1.1.209 3(or 17)alpha-hydroxysteroid dehydrogenase 3(or 17)alpha-hydroxysteroid:NAD(P)+ oxidoreductase
1.1.1.210 3beta(or 20alpha)-hydroxysteroid dehydrogenase 3beta(or 20alpha)-hydroxysteroid:NADP+ oxidoreductase
1.1.1.225 chlordecone reductase chlordecone-alcohol:NADP+ 2-oxidoreductase
1.1.1.270 3beta-hydroxysteroid 3-dehydrogenase 3beta-hydroxysteroid:NADP+ 3-oxidoreductase
1.1.1.357 3alpha-hydroxysteroid 3-dehydrogenase 3alpha-hydroxysteroid:NAD(P)+ 3-oxidoreductase
1.1.1.51 3(or 17)beta-hydroxysteroid dehydrogenase 3(or 17)beta-hydroxysteroid:NAD(P)+ oxidoreductase
1.1.1.53 3alpha(or 20beta)-hydroxysteroid dehydrogenase 3alpha(or 20beta)-hydroxysteroid:NAD+ oxidoreductase
1.1.1.62 17beta-estradiol 17-dehydrogenase 17beta-estradiol:NAD(P)+ 17-oxidoreductase
1.14.14.16 steroid 21-monooxygenase steroid,NADPH-hemoprotein reductase:oxygen oxidoreductase (21-hydroxylating)
1.14.14.19 steroid 17alpha-monooxygenase steroid,NADPH-hemoprotein reductase:oxygen oxidoreductase (17alpha-hydroxylating)
1.14.14.32 17alpha-hydroxyprogesterone deacetylase 17alpha-hydroxyprogesterone,NADPH-hemoprotein reductase:oxygen oxidoreductase (17alpha-hydroxylating, acetate-releasing)
1.14.15.4 steroid 11beta-monooxygenase steroid,reduced-adrenal-ferredoxin:oxygen oxidoreductase (11beta-hydroxylating)
1.14.15.5 corticosterone 18-monooxygenase corticosterone,reduced-adrenal-ferredoxin:oxygen oxidoreductase (18-hydroxylating)
1.14.15.6 cholesterol monooxygenase (side-chain-cleaving) cholesterol,reduced-adrenal-ferredoxin:oxygen oxidoreductase (side-chain-cleaving)
1.3.1.3 DELTA4-3-oxosteroid 5beta-reductase 5beta-cholestan-3-one:NADP+ 4,5-oxidoreductase
5.3.3.1 steroid DELTA-isomerase 3-oxosteroid DELTA5-DELTA4-isomerase


Proteins

Protein ID name full name