Entry Name Mitophagy - animal
Description Mitochondria act as the energy powerhouse of the cell, and are essential for eukaryotic cells to grow and function normally. However, deleterious byproducts of oxidative phosphorylation process called reactive oxidative species (ROS) lead to mitochondrial dysfunction. If the damage is too excessive to be repaired, such mitochondria are selectively recognized and targeted for degradation by a specific mode of autophagy, termed mitophagy. The loss of the mitochondrial membrane potential can induce mitophagy, involving the kinase PINK1 and the E3 ligase Parkin. PINK1 serves as the sensor for the mitochondrial depolarization and recruits Parkin, followed by ubiquitin-dependent recruitment of mitophagy receptors. There are also several PINK1/Parkin-independent mitophagy pathways, in which a group of LIR-containing receptors are required in response to different stimuli. Mitophagy contributes to the maintenance of a healthy mitochondrial network and the prevention of programmed cell death.
Class Cellular Processes; Transport and catabolismBRITE hierarchy
Pathway map Ortholog table Other DBs Orthology K17985 activating molecule in BECN1-regulated autophagy protein 1
K17771 mitochondrial import receptor subunit TOM7
K11518 mitochondrial import receptor subunit TOM40
K17770 mitochondrial import receptor subunit TOM20
K17768 mitochondrial import receptor subunit TOM70
K21356 mitofusin 1 [EC:3.6.5.-]
K06030 mitofusin 2 [EC:3.6.5.-]
K07870 mitochondrial Rho GTPase 1 [EC:3.6.5.-]
K07871 mitochondrial Rho GTPase 2 [EC:3.6.5.-]
K02927 ubiquitin-large subunit ribosomal protein L40e
K02977 ubiquitin-small subunit ribosomal protein S27Ae
K21348 calcium-binding and coiled-coil domain-containing protein 2
K17987 next to BRCA1 gene 1 protein
K08341 GABA(A) receptor-associated protein
K10435 microtubule-associated protein 1 light chain
K04570 Bcl-2-like 1 (apoptosis regulator Bcl-X)
K03115 casein kinase II subunit beta
K17986 FUN14 domain-containing protein 1
K15464 BCL2/adenovirus E1B 19 kDa protein-interacting protein 3
K15465 BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like
K07277 outer membrane protein insertion porin family
K08860 eukaryotic translation initiation factor 2-alpha kinase 3 [EC:2.7.11.1 ]
K03237 translation initiation factor 2 subunit 1
K04374 cyclic AMP-dependent transcription factor ATF-4
K04440 mitogen-activated protein kinase 8/9/10 (c-Jun N-terminal kinase) [EC:2.7.11.24 ]
K04448 transcription factor AP-1
K04735 transcription factor p65
K07831 Ras-related protein M-Ras
K07829 Ras-related protein R-Ras
K07830 Ras-related protein R-Ras2
K17454 transcription factor E2F1
K08268 hypoxia-inducible factor 1 alpha
K04684 transcription factor Sp1
K09408 forkhead box protein O3
K21361 Cbp/p300-interacting transactivator 2
K09455 microphthalmia-associated transcription factor
K15590 transcription factor EB
K09105 transcription factor E3
K20131 Rab5 GDP/GTP exchange factor
K07887 Ras-related protein Rab-5A
K07888 Ras-related protein Rab-5B
K07889 Ras-related protein Rab-5C
K20195 vacuolar fusion protein MON1
K27045 vacuolar fusion protein CCZ1, animal type
K07897 Ras-related protein Rab-7A
K07898 Ras-related protein Rab-7B
K20168 TBC1 domain family member 15
K19945 TBC1 domain family member 17
K17969 mitochondrial fission 1 protein
K17907 autophagy-related protein 9
K13525 transitional endoplasmic reticulum ATPase
Compound Reference Authors Pickrell AM, Youle RJ
Title The roles of PINK1, parkin, and mitochondrial fidelity in Parkinson's disease.
Journal Reference Authors Durcan TM, Fon EA
Title The three 'P's of mitophagy: PARKIN, PINK1, and post-translational modifications.
Journal Reference Authors Shirihai OS, Song M, Dorn GW 2nd
Title How mitochondrial dynamism orchestrates mitophagy.
Journal Reference Authors Hamacher-Brady A, Brady NR
Title Mitophagy programs: mechanisms and physiological implications of mitochondrial targeting by autophagy.
Journal Reference Authors Yamano K, Matsuda N, Tanaka K
Title The ubiquitin signal and autophagy: an orchestrated dance leading to mitochondrial degradation.
Journal Reference Authors Yamaguchi O, Murakawa T, Nishida K, Otsu K
Title Receptor-mediated mitophagy.
Journal Reference Authors Roberts RF, Tang MY, Fon EA, Durcan TM
Title Defending the mitochondria: The pathways of mitophagy and mitochondrial-derived vesicles.
Journal Reference Authors Arena G, Valente EM
Title PINK1 in the limelight: multiple functions of an eclectic protein in human health and disease.
Journal Related LinkDB All DBs
