Porphyria Variegata (PV)

Metabolites

5-Aminolevulinate

Formula: C5H9NO3 (131.0582404)

CAS ID: 106-60-5

Ammonia

Formula: NH3 (17.0265478)

CAS ID: 7664-41-7

Bilirubin

Formula: C33H36N4O6 (584.2634716)

CAS ID: 635-65-4

Biliverdin

Formula: C33H34N4O6 (582.2478224)

CAS ID: 114-25-0

CO

Formula: CO (27.994915)

CAS ID: 630-08-0

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

Coproporphyrinogen I

Formula: C36H44N4O8 (660.3158984)

CAS ID: 31110-56-2

Coproporphyrinogen III

Formula: C36H44N4O8 (660.3158984)

CAS ID: 2624-63-7

D-Urobilinogen

Formula: C33H42N4O6 (590.3104192000001)

CAS ID: 17208-65-0

Fe2+

Formula: Fe (55.934939)

CAS ID: 15438-31-0

Fe3+

Formula: Fe (55.934939)

CAS ID: 7439-89-6

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

Hydrogen peroxide

Formula: H2O2 (34.0054792)

CAS ID: 7722-84-1

Hydroxymethylbilane

Formula: C40H46N4O17 (854.2857826)

CAS ID: 71861-60-4

NAD+

Formula: C21H28N7O14P2 (664.1169428000001)

CAS ID: 53-84-9

NADH

Formula: C21H29N7O14P2 (665.1247674)

CAS ID: 58-68-4

Oxygen

Formula: O2 (31.98983)

CAS ID: 7782-44-7

Porphobilinogen

Formula: C10H14N2O4 (226.0953524)

CAS ID: 487-90-1

Protoporphyrinogen IX

Formula: C34H40N4O4 (568.30494)

CAS ID: 7412-77-3

UDP

Formula: C9H14N2O12P2 (404.0021984)

CAS ID: 58-98-0

UDP-glucuronate

Formula: C15H22N2O18P2 (580.0342852)

CAS ID: 2616-64-0

Uroporphyrinogen I

Formula: C40H44N4O16 (836.2752184)

CAS ID: 1867-62-5

Uroporphyrinogen III

Formula: C40H44N4O16 (836.2752184)

CAS ID: 1976-85-8



Enzyme

EC Number name full name note
1.14.14.18 heme oxygenase (biliverdin-producing) protoheme,NADPH-hemoprotein reductase:oxygen oxidoreductase (alpha-methene-oxidizing, hydroxylating)
1.16.3.1 ferroxidase Fe(II):oxygen oxidoreductase
1.17.99.9 heme a synthase ferroheme o:acceptor C-81-oxidoreductase (heme a-forming)
1.3.1.24 biliverdin reductase bilirubin:NAD(P)+ oxidoreductase
1.3.3.3 coproporphyrinogen oxidase coproporphyrinogen:oxygen oxidoreductase (decarboxylating)
1.3.3.4 protoporphyrinogen oxidase protoporphyrinogen-IX:oxygen oxidoreductase
2.3.1.37 5-aminolevulinate synthase succinyl-CoA:glycine C-succinyltransferase (decarboxylating)
2.4.1.17 glucuronosyltransferase UDP-glucuronate beta-D-glucuronosyltransferase (acceptor-unspecific)
2.5.1.141 heme o synthase (2E,6E)-farnesyl-diphosphate:protoheme IX farnesyltranstransferase
2.5.1.61 hydroxymethylbilane synthase porphobilinogen:(4-[2-carboxyethyl]-3-[carboxymethyl]pyrrol-2-yl)methyltransferase (hydrolysing)
3.2.1.31 beta-glucuronidase beta-D-glucuronoside glucuronosohydrolase
4.1.1.37 uroporphyrinogen decarboxylase uroporphyrinogen-III carboxy-lyase (coproporphyrinogen-III-forming)
4.2.1.24 porphobilinogen synthase 5-aminolevulinate hydro-lyase (adding 5-aminolevulinate and cyclizing; porphobilinogen-forming)
4.2.1.75 uroporphyrinogen-III synthase hydroxymethylbilane hydro-lyase (cyclizing; uroporphyrinogen-III-forming)
4.98.1.1 protoporphyrin ferrochelatase protoheme ferro-lyase (protoporphyrin-forming)


Proteins

Protein ID name full name