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The small inorganic Fe-S cluster (ISC) cofactor acts as an electron carrier in redox and catalytic reactions, and as a sulfur donor and environmental sensor. The ISC proteins can be found in mitochondria, cytoplasm and nucleus and are involved in energy production, tRNA modification and features of protein translation, DNA metabolism and repair. The steps in the assembly of iron-sulfur (Fe-S) clusters (ISC) and delivery to Fe-S proteins are part of the mitochondrial ISC biogenesis and the connec

Pathway Diagram:

Elsevier Inc. sulfide holo-Iscu Iscu Iba57 Nubpl Nfu1 mitochondrial iron-sulfur cluster export pathway Fe2+ holo-Iscu ---> Glrx5 Fdx1 ---> holo-Iscu Fdx1l ---> holo-Iscu Iscu ---- Nfs1 persulfide ---> sulfide Fe2+ ---> holo-Iscu Fdxr ---> Fdx1 Fdxr ---> Fdx1l holo-Iscu ---- Hspa9 holo-Iscu ---- Hscb Hspa9 Hscb ---- Hspa9 Fdxr Fdx1l Hscb Fdx1 Nfs1 Glrx5 ---- Hspa9 Lyrm4 ---- Nfs1 Lyrm4 Isca2 Isca1 ---- Isca2 Glrx5 Isca1 Glrx5 ---- Isca2 Glrx5 ---- Isca1 Bola3 persulfide

Genes in Pathway:

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mitochondrial iron-sulfur cluster protein biogenesis pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Bola3 bolA family member 3 JBrowse link 4 115,046,693 115,056,140 RGD:11554190
G Fdx1 ferredoxin 1 JBrowse link 8 56,373,729 56,393,199 RGD:11554190
G Fdx2 ferredoxin 2 JBrowse link 8 22,086,534 22,091,450 RGD:11554190
G Fdxr ferredoxin reductase JBrowse link 10 103,817,724 103,826,413 RGD:11554190
G Glrx5 glutaredoxin 5 JBrowse link 6 128,750,503 128,760,880 RGD:11554190
G Hscb HscB mitochondrial iron-sulfur cluster co-chaperone JBrowse link 12 51,878,267 51,888,645 RGD:11554190
G Hspa9 heat shock protein family A member 9 JBrowse link 18 27,731,072 27,749,235 RGD:11554190
G Iba57 IBA57, iron-sulfur cluster assembly JBrowse link 10 45,506,124 45,514,909 RGD:11554190
G Isca1 iron-sulfur cluster assembly 1 JBrowse link 17 5,281,727 5,294,386 RGD:11554190
G Isca2 iron-sulfur cluster assembly 2 JBrowse link 6 108,488,678 108,490,188 RGD:11554190
G Iscu iron-sulfur cluster assembly enzyme JBrowse link 12 48,621,454 48,627,297 RGD:11554190
G Lyrm4 LYR motif containing 4 JBrowse link 17 29,439,042 29,552,773 RGD:11554190
G Nfs1 NFS1 cysteine desulfurase JBrowse link 3 151,665,813 151,688,151 RGD:11554190
G Nfu1 NFU1 iron-sulfur cluster scaffold JBrowse link 4 118,814,284 118,834,955 RGD:11554190
G Nubpl nucleotide binding protein-like JBrowse link 6 72,891,758 73,147,837 RGD:11554190

Pathway Gene Annotations

Disease Annotations Associated with Genes in the mitochondrial iron-sulfur cluster protein biogenesis pathway
Disease TermsGene Symbols
Alzheimer's diseaseHspa9
Autoimmune DiseasesHspa9
autosomal dominant sideroblastic anemia 4Hspa9
autosomal recessive pyridoxine-refractory sideroblastic anemia 2Glrx5
autosomal recessive pyridoxine-refractory sideroblastic anemia 3Glrx5
brain ischemiaHspa9
combined oxidative phosphorylation deficiencyLyrm4
Combined Oxidative Phosphorylation Deficiency 14Lyrm4
Combined Oxidative Phosphorylation Deficiency 19Lyrm4
Developmental DisabilitiesIsca2
Failure to ThriveIsca2
genetic diseaseFdxr , Iba57 , Nubpl
hepatocellular carcinomaHspa9
Hereditary Neoplastic SyndromesHspa9
hereditary spastic paraplegia 74Iba57
human immunodeficiency virus infectious diseaseHspa9
intellectual disabilityIsca2
mitochondrial complex I deficiencyNubpl
Mitochondrial Complex I Deficiency, Nuclear Type 21Nubpl
mitochondrial metabolism diseaseNubpl
Multiple Mitochondrial Dysfunctions SyndromeBola3 , Iba57 , Isca1 , Isca2 , Nfu1
multiple mitochondrial dysfunctions syndrome 1Nfu1
multiple mitochondrial dysfunctions syndrome 2Bola3
multiple mitochondrial dysfunctions syndrome 3Iba57
multiple mitochondrial dysfunctions syndrome 4Isca2
multiple mitochondrial dysfunctions syndrome 5Isca1
Myocardial IschemiaHspa9
Myopathy with Lactic Acidosis, HereditaryIscu
Parkinson's diseaseHspa9
Parkinsonian DisordersHspa9
perinatal necrotizing enterocolitisHspa9
renal cell carcinomaHspa9
rheumatoid arthritisHspa9
sideroblastic anemiaGlrx5 , Hspa9
ventricular septal defectIsca2
Pathway Annotations Associated with Genes in the mitochondrial iron-sulfur cluster protein biogenesis pathway

References Associated with the mitochondrial iron-sulfur cluster protein biogenesis pathway:

Ontology Path Diagram:

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