nemoci-sympt/METABOLISMUS/mitochondrie/metabolizmus/fission
Fission
- Event of a single entity breaking apart [7]
- Fission is controlled by GTPases
- Driven by dynamin-related proteins
- DRP1
- OPA1 [8]
- Involved in the remodelling of cristae [8]
Fission
- Event of a single entity breaking apart [7]
- Fission is controlled by GTPases
- Driven by dynamin-related proteins
- DRP1
- OPA1 [8]
- Involved in the remodelling of cristae [8]
Fission and fusion
- Shape and mass are finely tuned by the activity of pro-fusion proteins
- Mitofusin 1 (MFN1)
- MFN2
- Optic atrophy protein 1 (OPA1)
- Pro-fission proteins
- Dynamin-related protein 1 (DRP1)
- mitochondrial fission 1 protein (FIS1) [9]
- Physiological balance between fission and fusion protects the integrity of mtDNA
- Continuous mixing of mtDNA pools [9]
Alterations in the genes encoding these complex machineries lead to disease
- Mutations in OPA1
- Autosomal dominant optic atrophy [9]
- Mutations in MFN2
- Charcot–Marie–Tooth disease type 2A [9]
- Disruption of Mfn1 and Mfn2 in the skeletal muscle of the POLGD257A mutator mouse
- Striking worsening of the phenotype
- Accumulation of mtDNA mutations [9]
- Overexpression of Opa1
- Multitasking GTPase
- Involved in shaping mitochondrial cristae
- Promoting fusion of the inner mitochondrial membrane
- Increase respiratory efficiency
- By stabilizing the respiratory chain supercomplexes [9]
- Some compounds affecting fission and fusion have been identified
- Drp1 inhibitor MDIVI-1
- M1-hydrazone
- Probably promotes fusion
- By acting on Mfn or Opa1 [9]
Fission and fusion
- Shape and mass are finely tuned by the activity of pro-fusion proteins
- Mitofusin 1 (MFN1)
- MFN2
- Optic atrophy protein 1 (OPA1)
- Pro-fission proteins
- Dynamin-related protein 1 (DRP1)
- mitochondrial fission 1 protein (FIS1) [9]
- Physiological balance between fission and fusion protects the integrity of mtDNA
- Continuous mixing of mtDNA pools [9]
Alterations in the genes encoding these complex machineries lead to disease
- Mutations in OPA1
- Autosomal dominant optic atrophy [9]
- Mutations in MFN2
- Charcot–Marie–Tooth disease type 2A [9]
- Disruption of Mfn1 and Mfn2 in the skeletal muscle of the POLGD257A mutator mouse
- Striking worsening of the phenotype
- Accumulation of mtDNA mutations [9]
- Overexpression of Opa1
- Multitasking GTPase
- Involved in shaping mitochondrial cristae
- Promoting fusion of the inner mitochondrial membrane
- Increase respiratory efficiency
- By stabilizing the respiratory chain supercomplexes [9]
- Some compounds affecting fission and fusion have been identified
- Drp1 inhibitor MDIVI-1
- M1-hydrazone
- Probably promotes fusion
- By acting on Mfn or Opa1 [9]
