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Proteins/UNIPROT:P22570
Serves as the first electron transfer protein in all the mitochondrial P450 systems including cholesterol side chain cleavage in all steroidogenic tissues, steroid 11-beta hydroxylation in the adrenal cortex, 25-OH-vitamin D3-24 hydroxylation in the kidney, and sterol C-27 hydroxylation in the liver (By similarity). Also acts as a ferredoxin--NADP(+) reductase essential for coenzyme Q biosynthesis: together with FDX2, transfers the electrons required for the hydroxylation reaction performed by COQ6..
Function from UniProtKB (P22570), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 2 · substrate of
2 compounds
2 compounds.
0 reported routes2 assembled routes
3 rows
83 statements
83 statements · 5 reference databases · Subject: FDXR · Sources: Hetionet, Human-GEM, Reactome, UniProt, WikiPathways
6 reactions
MAM00210m+3proton+NADPH(4-)+2S-adenosyl-L-methionine+tetra-mu3-sulfido-tetrairon(2+)MAM00208m+2MAM01098m+4iron(2+)+2hydrogen sulfide+2L-methionine+NADP(3-)
5 names
Resolves to: FDXR
Reaction pageLeft to rightBalancedEC 2.8.1.8mitochondrial matrix
Human-GEM MAR06403CC BY 4.0Reference data
proton+NADP(3-)+2MAM02828mNADPH(4-)+2MAM02665m
Catalysed by FDXR
Reaction pageReversibleBalance undeterminableEC 1.18.1.2mitochondrial matrix
Human-GEM MAR07741CC BY 4.0Reference data
2ATP+2water+NADPH(4-)+2di-mu-sulfido-diiron(2+)2ADP+3proton+NADP(3-)+2phosphate(3-)+tetra-mu3-sulfido-tetrairon(2+)
Catalysed by FDXR & HSPA9 & IBA57 & FDX2 & GLRX5 & ISCA2 & HSCB & ISCA1
Reaction pageLeft to rightBalancedmitochondrial matrix
Human-GEM MAR20184CC BY 4.0Reference data
2L-cysteine+2iron(2+)+NADPH(4-)2L-alanine+proton+NADP(3-)+di-mu-sulfido-diiron(2+)
Catalysed by NDUFAB1 & FDXR & FXN & FDX2 & ISCU & LYRM4 & NFS1
Reaction pageLeft to rightBalancedmitochondrial matrix
Human-GEM MAR20185CC BY 4.0Reference data
2reduced [2Fe-2S]-[ferredoxin]+NADP(3-)+hydron2oxidized [2Fe-2S]-[ferredoxin]+NADPH(4-)
Catalysed by FDXR
Reaction pageDirection not assertedBalance undeterminable
Rhea RHEA:20125CC BY 4.0Reference data
2reduced [adrenodoxin]+NADP(3-)+hydron2oxidized [adrenodoxin]+NADPH(4-)
Catalysed by FDXR
Reaction pageDirection not assertedBalance undeterminable
Rhea RHEA:42312CC BY 4.0Reference data