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| HMBS | Porphobilinogen deaminase; Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps. (361 aa) | ||||
| COX10 | Protoheme IX farnesyltransferase, mitochondrial; Converts protoheme IX and farnesyl diphosphate to heme O. Belongs to the ubiA prenyltransferase family. (443 aa) | ||||
| BDH2 | 3-hydroxybutyrate dehydrogenase type 2; Dehydrogenase that mediates the formation of 2,5- dihydroxybenzoic acid (2,5-DHBA), a siderophore that shares structural similarities with bacterial enterobactin and associates with LCN2, thereby playing a key role in iron assimilation and homeostasis. Plays a role in susceptibility to bacterial infection by providing an assimilable source of iron that is exploited by pathogenic bacteria (By similarity). Also acts as a 3-hydroxybutyrate dehydrogenase (By similarity). (245 aa) | ||||
| BLVRA | Biliverdin reductase A; Reduces the gamma-methene bridge of the open tetrapyrrole, biliverdin IX alpha, to bilirubin with the concomitant oxidation of a NADH or NADPH cofactor; Belongs to the Gfo/Idh/MocA family. Biliverdin reductase subfamily. (296 aa) | ||||
| BLVRB | Flavin reductase (NADPH); Broad specificity oxidoreductase that catalyzes the NADPH- dependent reduction of a variety of flavins, such as riboflavin, FAD or FMN, biliverdins, methemoglobin and PQQ (pyrroloquinoline quinone). Contributes to heme catabolism and metabolizes linear tetrapyrroles. Can also reduce the complexed Fe(3+) iron to Fe(2+) in the presence of FMN and NADPH. In the liver, converts biliverdin to bilirubin. (206 aa) | ||||
| FECH | Ferrochelatase, mitochondrial; Catalyzes the ferrous insertion into protoporphyrin IX. (416 aa) | ||||
| ATP5IF1 | ATPase inhibitor, mitochondrial; Endogenous F(1)F(o)-ATPase inhibitor limiting ATP depletion when the mitochondrial membrane potential falls below a threshold and the F(1)F(o)-ATP synthase starts hydrolyzing ATP to pump protons out of the mitochondrial matrix. Required to avoid the consumption of cellular ATP when the F(1)F(o)-ATP synthase enzyme acts as an ATP hydrolase. (109 aa) | ||||
| TMEM14C | Transmembrane protein 14C; Required for normal heme biosynthesis. (114 aa) | ||||
| UROS | Uroporphyrinogen III synthase. (264 aa) | ||||
| CPOX | Coproporphyrinogen oxidase. (449 aa) | ||||
| ALAS1 | 5-aminolevulinate synthase, nonspecific, mitochondrial; Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family. (752 aa) | ||||
| HPX | Hemopexin; Binds heme and transports it to the liver for breakdown and iron recovery, after which the free hemopexin returns to the circulation. (459 aa) | ||||
| COX15 | Cytochrome c oxidase assembly protein COX15 homolog; May be involved in the biosynthesis of heme A; Belongs to the COX15/CtaA family. (426 aa) | ||||
| ALAD | Delta-aminolevulinic acid dehydratase; Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen (By similarity); Belongs to the ALAD family. (329 aa) | ||||
| FXN | Frataxin intermediate form; Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization. Modulates the RNA-binding activity of ACO1 (By similarity). (217 aa) | ||||
| ENSBTAP00000067738 | Uncharacterized protein. (156 aa) | ||||
| ALAS2 | 5-aminolevulinate synthase, erythroid-specific, mitochondrial; Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family. (613 aa) | ||||
| HMOX1 | Heme oxygenase 1; Heme oxygenase cleaves the heme ring at the alpha methene bridge to form biliverdin. Biliverdin is subsequently converted to bilirubin by biliverdin reductase. Under physiological conditions, the activity of heme oxygenase is highest in the spleen, where senescent erythrocytes are sequestrated and destroyed. Exhibits cytoprotective effects since excess of free heme sensitizes cells to undergo apoptosis. (302 aa) | ||||
| UROD | Uroporphyrinogen decarboxylase; Belongs to the uroporphyrinogen decarboxylase family. (367 aa) | ||||
| IREB2 | Iron responsive element binding protein 2; Belongs to the aconitase/IPM isomerase family. (964 aa) | ||||
| G3MXH9_BOVIN | Uncharacterized protein. (144 aa) | ||||
| PPOX | Protoporphyrinogen oxidase; Catalyzes the 6-electron oxidation of protoporphyrinogen-IX to form protoporphyrin-IX. (477 aa) | ||||
| HMOX2 | Heme oxygenase 2. (334 aa) | ||||
| HNF1A | HNF1 homeobox A. (633 aa) | ||||
| ABCB6 | ATP binding cassette subfamily B member 6. (843 aa) | ||||
| PGRMC1 | Membrane-associated progesterone receptor component 1; Component of a progesterone-binding protein complex. Binds progesterone. Has many reported cellular functions (heme homeostasis, interaction with CYPs). (230 aa) | ||||
| SLC25A39 | Solute carrier family 25 member 39; Required for normal heme biosynthesis. (359 aa) | ||||
| ABCB10 | ATP binding cassette subfamily B member 10. (433 aa) | ||||