Entry Name Mitophagy - animal - Rhinopithecus bieti (black snub-nosed monkey)
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 Rhinopithecus bieti (black snub-nosed monkey) [GN:
rbb ]
Gene 108512512 MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435 ]
108514005 SAMM50; sorting and assembly machinery component 50 homolog [KO:K07277 ]
108514304 GABARAP; gamma-aminobutyric acid receptor-associated protein isoform X1 [KO:K08341 ]
108516482 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2 isoform X1 [KO:K21348 ]
108518882 EIF2AK3; LOW QUALITY PROTEIN: eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860 ] [EC:2.7.11.1 ]
108519441 RHOT2; mitochondrial Rho GTPase 2 isoform X1 [KO:K07871 ] [EC:3.6.5.-]
108520463 ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
108520768 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435 ]
108521188 TOMM40L; mitochondrial import receptor subunit TOM40B [KO:K11518 ]
108522209 TOMM20; mitochondrial import receptor subunit TOM20 homolog [KO:K17770 ]
108523547 MITF; microphthalmia-associated transcription factor isoform X1 [KO:K09455 ]
108524470 VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525 ]
108526974 MON1A; vacuolar fusion protein MON1 homolog A isoform X1 [KO:K20195 ]
108527406 MON1B; LOW QUALITY PROTEIN: vacuolar fusion protein MON1 homolog B [KO:K20195 ]
108527415 GABARAPL2; gamma-aminobutyric acid receptor-associated protein-like 2 [KO:K08341 ]
108529328 TOMM7; mitochondrial import receptor subunit TOM7 homolog [KO:K17771 ]
108531118 TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518 ]
108533712 EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
108533773 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435 ]
108534205 UBA52; ubiquitin-60S ribosomal protein L40 isoform X1 [KO:K02927 ]
108537689 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like isoform X1 [KO:K15465 ]
108541403 RHOT1; mitochondrial Rho GTPase 1 isoform X1 [KO:K07870 ] [EC:3.6.5.-]
108542138 GABARAPL1; gamma-aminobutyric acid receptor-associated protein-like 1 [KO:K08341 ]
108543388 BNIP3; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 [KO:K15464 ]
108543840 RABGEF1; LOW QUALITY PROTEIN: rab5 GDP/GTP exchange factor [KO:K20131 ]
108544440 HIF1A; LOW QUALITY PROTEIN: hypoxia-inducible factor 1-alpha [KO:K08268 ]
108544496 PGAM5; LOW QUALITY PROTEIN: serine/threonine-protein phosphatase PGAM5, mitochondrial [KO:K15637 ] [EC:3.1.3.16 ]
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
