FXN protein (human) - STRING database

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FXN

Frataxin, mitochondrial; 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; however, the physiological relevance is unsure as reports are conflicting and the function has only [...] (210 aa)

Predicted Functional Partners:

ISCU

0.997

LYRM4

0.997

NFS1

0.996

PMPCB

0.994

HSCB

0.993

ACO2

0.991

FECH

0.988

HSPA9

0.987

FDX1L

0.981

FDX1

0.978

Your Current Organism:

Homo sapiens

NCBI taxonomy Id: 9606
Other names: H. sapiens, Homo sapiens, human, man

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Browse interactions in tabular form:

node1	node2	node1 accession	node2 accession	node1 annotation	node2 annotation	score
ACO2	FDX1L	ENSP00000216254	ENSP00000377311	Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family	Ferredoxin-2, mitochondrial; Essential for heme A and Fe/S protein biosynthesis; Belongs to the adrenodoxin/putidaredoxin family	0.532
ACO2	FECH	ENSP00000216254	ENSP00000372326	Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family	Ferrochelatase, mitochondrial; Catalyzes the ferrous insertion into protoporphyrin IX; Belongs to the ferrochelatase family	0.763
ACO2	FXN	ENSP00000216254	ENSP00000366482	Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family	Frataxin, mitochondrial; 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; however, the physiological relevance is unsure as reports are conflicting and the function has only [...]	0.991
ACO2	HSCB	ENSP00000216254	ENSP00000216027	Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family	Iron-sulfur cluster co-chaperone protein HscB, mitochondrial; Acts as a co-chaperone in iron-sulfur cluster assembly in mitochondria; Belongs to the HscB family	0.944
ACO2	HSPA9	ENSP00000216254	ENSP00000297185	Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family	Stress-70 protein, mitochondrial; Chaperone protein which plays an important role in mitochondrial iron-sulfur cluster (ISC) biogenesis. Interacts with and stabilizes ISC cluster assembly proteins FXN, NFU1, NFS1 and ISCU. Regulates erythropoiesis via stabilization of ISC assembly. May play a role in the control of cell proliferation and cellular aging (By similarity); Belongs to the heat shock protein 70 family	0.961
ACO2	ISCU	ENSP00000216254	ENSP00000310623	Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family	Iron-sulfur cluster assembly enzyme ISCU, mitochondrial; Scaffold protein for the de novo synthesis of iron- sulfur (Fe-S) clusters within mitochondria, which is required for maturation of both mitochondrial and cytoplasmic [2Fe-2S] and [4Fe-4S] proteins. First, a [2Fe-2S] cluster is transiently assembled on the scaffold protein ISCU. In a second step, the cluster is released from ISCU, transferred to a glutaredoxin GLRX5, followed by the formation of mitochondrial [2Fe-2S] proteins, the synthesis of [4Fe-4S] clusters and their target-specific insertion into the recipient apoproteins. [...]	0.798
ACO2	LYRM4	ENSP00000216254	ENSP00000420026	Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family	LYR motif-containing protein 4; Required for nuclear and mitochondrial iron-sulfur protein biosynthesis; LYR motif containing	0.502
ACO2	NFS1	ENSP00000216254	ENSP00000363205	Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family	Cysteine desulfurase, mitochondrial; Catalyzes the removal of elemental sulfur from cysteine to produce alanine. It supplies the inorganic sulfur for iron- sulfur (Fe-S) clusters. May be involved in the biosynthesis of molybdenum cofactor	0.654
ACO2	PMPCB	ENSP00000216254	ENSP00000249269	Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family	Mitochondrial-processing peptidase subunit beta; Cleaves presequences (transit peptides) from mitochondrial protein precursors; M16 metallopeptidases	0.594
FDX1	FDX1L	ENSP00000260270	ENSP00000377311	Adrenodoxin, mitochondrial; Participates in the synthesis of thyroid hormones. Essential for the synthesis of various steroid hormones, participates in the reduction of mitochondrial cytochrome P450 for steroidogenesis. Transfers electrons from adrenodoxin reductase to CYP11A1, a cytochrome P450 that catalyzes cholesterol side-chain cleavage; Belongs to the adrenodoxin/putidaredoxin family	Ferredoxin-2, mitochondrial; Essential for heme A and Fe/S protein biosynthesis; Belongs to the adrenodoxin/putidaredoxin family	0.949
FDX1	FECH	ENSP00000260270	ENSP00000372326	Adrenodoxin, mitochondrial; Participates in the synthesis of thyroid hormones. Essential for the synthesis of various steroid hormones, participates in the reduction of mitochondrial cytochrome P450 for steroidogenesis. Transfers electrons from adrenodoxin reductase to CYP11A1, a cytochrome P450 that catalyzes cholesterol side-chain cleavage; Belongs to the adrenodoxin/putidaredoxin family	Ferrochelatase, mitochondrial; Catalyzes the ferrous insertion into protoporphyrin IX; Belongs to the ferrochelatase family	0.502
FDX1	FXN	ENSP00000260270	ENSP00000366482	Adrenodoxin, mitochondrial; Participates in the synthesis of thyroid hormones. Essential for the synthesis of various steroid hormones, participates in the reduction of mitochondrial cytochrome P450 for steroidogenesis. Transfers electrons from adrenodoxin reductase to CYP11A1, a cytochrome P450 that catalyzes cholesterol side-chain cleavage; Belongs to the adrenodoxin/putidaredoxin family	Frataxin, mitochondrial; 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; however, the physiological relevance is unsure as reports are conflicting and the function has only [...]	0.978
FDX1	HSCB	ENSP00000260270	ENSP00000216027	Adrenodoxin, mitochondrial; Participates in the synthesis of thyroid hormones. Essential for the synthesis of various steroid hormones, participates in the reduction of mitochondrial cytochrome P450 for steroidogenesis. Transfers electrons from adrenodoxin reductase to CYP11A1, a cytochrome P450 that catalyzes cholesterol side-chain cleavage; Belongs to the adrenodoxin/putidaredoxin family	Iron-sulfur cluster co-chaperone protein HscB, mitochondrial; Acts as a co-chaperone in iron-sulfur cluster assembly in mitochondria; Belongs to the HscB family	0.926
FDX1	HSPA9	ENSP00000260270	ENSP00000297185	Adrenodoxin, mitochondrial; Participates in the synthesis of thyroid hormones. Essential for the synthesis of various steroid hormones, participates in the reduction of mitochondrial cytochrome P450 for steroidogenesis. Transfers electrons from adrenodoxin reductase to CYP11A1, a cytochrome P450 that catalyzes cholesterol side-chain cleavage; Belongs to the adrenodoxin/putidaredoxin family	Stress-70 protein, mitochondrial; Chaperone protein which plays an important role in mitochondrial iron-sulfur cluster (ISC) biogenesis. Interacts with and stabilizes ISC cluster assembly proteins FXN, NFU1, NFS1 and ISCU. Regulates erythropoiesis via stabilization of ISC assembly. May play a role in the control of cell proliferation and cellular aging (By similarity); Belongs to the heat shock protein 70 family	0.605
FDX1	ISCU	ENSP00000260270	ENSP00000310623	Adrenodoxin, mitochondrial; Participates in the synthesis of thyroid hormones. Essential for the synthesis of various steroid hormones, participates in the reduction of mitochondrial cytochrome P450 for steroidogenesis. Transfers electrons from adrenodoxin reductase to CYP11A1, a cytochrome P450 that catalyzes cholesterol side-chain cleavage; Belongs to the adrenodoxin/putidaredoxin family	Iron-sulfur cluster assembly enzyme ISCU, mitochondrial; Scaffold protein for the de novo synthesis of iron- sulfur (Fe-S) clusters within mitochondria, which is required for maturation of both mitochondrial and cytoplasmic [2Fe-2S] and [4Fe-4S] proteins. First, a [2Fe-2S] cluster is transiently assembled on the scaffold protein ISCU. In a second step, the cluster is released from ISCU, transferred to a glutaredoxin GLRX5, followed by the formation of mitochondrial [2Fe-2S] proteins, the synthesis of [4Fe-4S] clusters and their target-specific insertion into the recipient apoproteins. [...]	0.956
FDX1	LYRM4	ENSP00000260270	ENSP00000420026	Adrenodoxin, mitochondrial; Participates in the synthesis of thyroid hormones. Essential for the synthesis of various steroid hormones, participates in the reduction of mitochondrial cytochrome P450 for steroidogenesis. Transfers electrons from adrenodoxin reductase to CYP11A1, a cytochrome P450 that catalyzes cholesterol side-chain cleavage; Belongs to the adrenodoxin/putidaredoxin family	LYR motif-containing protein 4; Required for nuclear and mitochondrial iron-sulfur protein biosynthesis; LYR motif containing	0.977
FDX1	NFS1	ENSP00000260270	ENSP00000363205	Adrenodoxin, mitochondrial; Participates in the synthesis of thyroid hormones. Essential for the synthesis of various steroid hormones, participates in the reduction of mitochondrial cytochrome P450 for steroidogenesis. Transfers electrons from adrenodoxin reductase to CYP11A1, a cytochrome P450 that catalyzes cholesterol side-chain cleavage; Belongs to the adrenodoxin/putidaredoxin family	Cysteine desulfurase, mitochondrial; Catalyzes the removal of elemental sulfur from cysteine to produce alanine. It supplies the inorganic sulfur for iron- sulfur (Fe-S) clusters. May be involved in the biosynthesis of molybdenum cofactor	0.885
FDX1L	ACO2	ENSP00000377311	ENSP00000216254	Ferredoxin-2, mitochondrial; Essential for heme A and Fe/S protein biosynthesis; Belongs to the adrenodoxin/putidaredoxin family	Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis-aconitate; Belongs to the aconitase/IPM isomerase family	0.532
FDX1L	FDX1	ENSP00000377311	ENSP00000260270	Ferredoxin-2, mitochondrial; Essential for heme A and Fe/S protein biosynthesis; Belongs to the adrenodoxin/putidaredoxin family	Adrenodoxin, mitochondrial; Participates in the synthesis of thyroid hormones. Essential for the synthesis of various steroid hormones, participates in the reduction of mitochondrial cytochrome P450 for steroidogenesis. Transfers electrons from adrenodoxin reductase to CYP11A1, a cytochrome P450 that catalyzes cholesterol side-chain cleavage; Belongs to the adrenodoxin/putidaredoxin family	0.949
FDX1L	FECH	ENSP00000377311	ENSP00000372326	Ferredoxin-2, mitochondrial; Essential for heme A and Fe/S protein biosynthesis; Belongs to the adrenodoxin/putidaredoxin family	Ferrochelatase, mitochondrial; Catalyzes the ferrous insertion into protoporphyrin IX; Belongs to the ferrochelatase family	0.456

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