Entry Name Mitophagy - animal - Trachypithecus francoisi (Francois's langur)
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 Trachypithecus francoisi (Francois's langur) [GN:
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Gene 117062294 RPS27A; LOW QUALITY PROTEIN: ubiquitin-40S ribosomal protein S27a [KO:K02977 ]
117067175 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2 [KO:K21348 ]
117067548 RHOT1; mitochondrial Rho GTPase 1 isoform X1 [KO:K07870 ] [EC:3.6.5.-]
117067806 GABARAP; gamma-aminobutyric acid receptor-associated protein [KO:K08341 ]
117068677 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435 ]
117069444 EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
117072566 TOMM7; mitochondrial import receptor subunit TOM7 homolog [KO:K17771 ]
117074638 TOMM40L; mitochondrial import receptor subunit TOM40B [KO:K11518 ]
117074955 MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435 ]
117076137 TOMM20; mitochondrial import receptor subunit TOM20 homolog [KO:K17770 ]
117079889 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985 ]
117081715 MON1A; vacuolar fusion protein MON1 homolog A isoform X1 [KO:K20195 ]
117082566 BNIP3; LOW QUALITY PROTEIN: BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 [KO:K15464 ]
117083122 MITF; microphthalmia-associated transcription factor isoform X1 [KO:K09455 ]
117088066 GABARAPL2; gamma-aminobutyric acid receptor-associated protein-like 2 isoform X1 [KO:K08341 ]
117088081 MON1B; vacuolar fusion protein MON1 homolog B isoform X1 [KO:K20195 ]
117088175 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435 ]
117090562 GABARAPL1; gamma-aminobutyric acid receptor-associated protein-like 1 isoform X1 [KO:K08341 ]
117090963 MAP1LC3B2; LOW QUALITY PROTEIN: microtubule-associated proteins 1A/1B light chain 3 beta 2 [KO:K10435 ]
117091371 TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518 ]
117093685 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like isoform X1 [KO:K15465 ]
117096351 SAMM50; sorting and assembly machinery component 50 homolog isoform X1 [KO:K07277 ]
117096463 ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
117097896 RHOT2; mitochondrial Rho GTPase 2 isoform X1 [KO:K07871 ] [EC:3.6.5.-]
117099028 VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525 ]
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
