Entry Name Mitophagy - animal - Delphinapterus leucas (beluga whale)
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 Delphinapterus leucas (beluga whale) [GN:
dle ]
Gene 111163933 TOMM20; mitochondrial import receptor subunit TOM20 homolog [KO:K17770 ]
111166325 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2 [KO:K21348 ]
111167070 TOMM40L; mitochondrial import receptor subunit TOM40B isoform X1 [KO:K11518 ]
111172181 BNIP3; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 [KO:K15464 ]
111173047 EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
111174194 MITF; microphthalmia-associated transcription factor isoform X1 [KO:K09455 ]
111174375 MON1A; vacuolar fusion protein MON1 homolog A isoform X1 [KO:K20195 ]
111176756 GABARAPL1; gamma-aminobutyric acid receptor-associated protein-like 1 [KO:K08341 ]
111177426 MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435 ]
111179417 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435 ]
111179501 GABARAPL2; gamma-aminobutyric acid receptor-associated protein-like 2 isoform X1 [KO:K08341 ]
111180759 TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518 ]
111181894 CCZ1; vacuolar fusion protein CCZ1 homolog isoform X1 [KO:K27045 ]
111182006 TOMM7; mitochondrial import receptor subunit TOM7 homolog isoform X1 [KO:K17771 ]
111182242 TOMM70; mitochondrial import receptor subunit TOM70 isoform X1 [KO:K17768 ]
111183200 ATG9B; LOW QUALITY PROTEIN: autophagy-related protein 9B [KO:K17907 ]
111183981 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985 ]
111184255 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435 ]
111185112 RHOT2; mitochondrial Rho GTPase 2 isoform X1 [KO:K07871 ] [EC:3.6.5.-]
111185418 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like isoform X1 [KO:K15465 ]
111185712 GABARAP; gamma-aminobutyric acid receptor-associated protein [KO:K08341 ]
111185854 RHOT1; mitochondrial Rho GTPase 1 isoform X1 [KO:K07870 ] [EC:3.6.5.-]
111186212 ATF4; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374 ]
111186276 SAMM50; sorting and assembly machinery component 50 homolog [KO:K07277 ]
111188117 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
