Entry Name Mitophagy - animal - Theropithecus gelada (gelada)
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 Other DBs Organism Theropithecus gelada (gelada) [GN:
tge ]
Gene 112606606 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 [KO:K17985 ]
112607814 VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525 ]
112608982 GABARAP; gamma-aminobutyric acid receptor-associated protein isoform X1 [KO:K08341 ]
112608984 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2 isoform X1 [KO:K21348 ]
112609434 RHOT1; mitochondrial Rho GTPase 1 isoform X1 [KO:K07870 ] [EC:3.6.5.-]
112610002 MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435 ]
112612007 UBA52; ubiquitin-60S ribosomal protein L40 isoform X1 [KO:K02927 ]
112612928 TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518 ]
112613697 GABARAPL2; gamma-aminobutyric acid receptor-associated protein-like 2 [KO:K08341 ]
112614047 RHOT2; mitochondrial Rho GTPase 2 isoform X1 [KO:K07871 ] [EC:3.6.5.-]
112614142 MON1B; vacuolar fusion protein MON1 homolog B isoform X1 [KO:K20195 ]
112614444 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B isoform X1 [KO:K10435 ]
112618707 MITF; microphthalmia-associated transcription factor isoform X1 [KO:K09455 ]
112619108 MON1A; vacuolar fusion protein MON1 homolog A isoform X1 [KO:K20195 ]
112620360 ATG9B; LOW QUALITY PROTEIN: autophagy-related protein 9B [KO:K17907 ]
112620487 TOMM7; mitochondrial import receptor subunit TOM7 homolog [KO:K17771 ]
112623249 CITED2; cbp/p300-interacting transactivator 2 isoform X1 [KO:K21361 ]
112629021 EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
112630426 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like isoform X1 [KO:K15465 ]
112631172 BNIP3; LOW QUALITY PROTEIN: BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 [KO:K15464 ]
112631202 TOMM40L; mitochondrial import receptor subunit TOM40B isoform X1 [KO:K11518 ]
112632664 ATF4; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374 ]
112632741 SAMM50; sorting and assembly machinery component 50 homolog [KO:K07277 ]
112633328 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A isoform X1 [KO:K10435 ]
112633963 GABARAPL1; gamma-aminobutyric acid receptor-associated protein-like 1 isoform X1 [KO:K08341 ]
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 KO pathway
