Mitochondrial fatty acid synthesis and respiration (Homo sapiens)

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1, 2MFN2BNIP3Mitochondrial fusionFIS1MIB1MFN1ProhibitinOPA1MFN1MFN2BNIP3DRP1DRP1FIS1BAXMitochondrial fissionBCL2Endophilin-A1PDLMTP18OPA1


"The dynamics of mitochondrial fission and fusion. The localization, as well as some interaction and modification of the principal proteins involved in the two processes are shown. Once dephosphorylated, DRP1 is recruited to the outer membrane by FIS1 or by another, unknown, component. The oligomerization of DRP1 is followed by constriction of the membrane and mitochondrial fission. The pro-fusion proteins (MFNs on the outer membrane and OPA1 on the inner membrane) oligomerize to induce fusion of the membranes. Other additional components of the machinery are shown. BAX, BCL2-associated X protein; BNIP3, BCL2/E1B 19 kDa-interacting protein 3; CAMK1a, calcium/calmodulin-dependent protein kinase 1a; DRP1, dynamin-related protein 1; FIS1, fission protein 1; GDAP1, ganglioside-induced differentiation-associated protein 1; l-OPA1, long form of OPA1; MFN, mitofusin; MIB, mitofusin-binding protein; MTP18, mitochondrial protein 18 kDa; OPA1, optic atrophy 1; PKA, protein kinase A; PLD, phospholipase D; s‑OPA1: short form of OPA1." Luca Scorrano et al 2010

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Ontology Terms



  1. Hiltunen JK, Autio KJ, Schonauer MS, Kursu VA, Dieckmann CL, Kastaniotis AJ; ''Mitochondrial fatty acid synthesis and respiration.''; Biochim Biophys Acta, 2010 PubMed Europe PMC Scholia
  2. Hiltunen JK, Schonauer MS, Autio KJ, Mittelmeier TM, Kastaniotis AJ, Dieckmann CL; ''Mitochondrial fatty acid synthesis type II: more than just fatty acids.''; J Biol Chem, 2009 PubMed Europe PMC Scholia


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109037view18:17, 14 February 2020KhanspersOntology Term : 'mitochondria fusion pathway' added !
109036view18:17, 14 February 2020KhanspersOntology Term : 'mitochondria fission pathway' added !
109035view18:16, 14 February 2020KhanspersOntology Term : 'PW:0002075' removed !
109034view18:16, 14 February 2020KhanspersOntology Term : 'altered mitochondria fission pathway' added !
97926view08:50, 25 June 2018MadeomugaModified description
97925view08:47, 25 June 2018MadeomugaNew pathway

External references


View all...
NameTypeDatabase referenceComment
BAXGeneProductENSG00000087088 (Ensembl)
BCL2GeneProductENSG00000171552 (Ensembl)
BNIP3GeneProductENSG00000176171 (Ensembl)
DRP1 GeneProductENSG00000087470 (Ensembl)
Endophilin-A1GeneProductENSG00000107295 (Ensembl)
FIS1GeneProductENSG00000214253 (Ensembl)
MFN1GeneProductENSG00000171109 (Ensembl)
MFN2GeneProductENSG00000116688 (Ensembl)
MIB1GeneProductENSG00000101752 (Ensembl)
MTP18 GeneProductENSG00000242114 (Ensembl)
Mitochondrial fissionPathway
Mitochondrial fusionPathway
OPA1GeneProductENSG00000198836 (Ensembl)
PDLGeneProductENSG00000075651 (Ensembl)
ProhibitinProteinA8K401 (Uniprot-TrEMBL)

Annotated Interactions

No annotated interactions

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