Entry Name Mitophagy - animal - Onychostoma macrolepis
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 Onychostoma macrolepis [GN:
omc ]
Gene 131520731 hif1al; hypoxia inducible factor 1 subunit alpha, like isoform X1 [KO:K08268 ]
131520946 rabgef1l; RAB guanine nucleotide exchange factor (GEF) 1, like [KO:K20131 ]
131521275 tax1bp1b; tax1-binding protein 1 homolog B isoform X1 [KO:K21347 ]
131522293 zgc:92606; gamma-aminobutyric acid receptor-associated protein-like 1 [KO:K08341 ]
131522437 tomm40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518 ]
131522788 mul1a; LOW QUALITY PROTEIN: mitochondrial ubiquitin ligase activator of nfkb 1-A [KO:K15688 ] [EC:2.3.2.27 ]
131523114 eif2s1a; eukaryotic translation initiation factor 2 subunit 1a [KO:K03237 ]
131525321 tomm7; mitochondrial import receptor subunit TOM7 homolog [KO:K17771 ]
131526395 tomm40l; mitochondrial import receptor subunit TOM40B [KO:K11518 ]
131526543 eif2s1b; eukaryotic translation initiation factor 2 subunit 1b [KO:K03237 ]
131531957 mitfb; melanocyte inducing transcription factor b isoform X1 [KO:K09455 ]
131533979 map1lc3b; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435 ]
131534009 gabarapl2; gamma-aminobutyric acid receptor-associated protein-like 2 [KO:K08341 ]
131534055 ambra1b; activating molecule in BECN1-regulated autophagy protein 1B [KO:K17985 ]
131534178 samm50; sorting and assembly machinery component 50 homolog A [KO:K07277 ]
131536834 rhot1a; mitochondrial Rho GTPase 1-A isoform X1 [KO:K07870 ] [EC:3.6.5.-]
131537025 tbc1d17; LOW QUALITY PROTEIN: TBC1 domain family member 17 [KO:K19945 ]
131539014 samm50l; sorting and assembly machinery component 50 homolog B [KO:K07277 ]
131539526 zgc:136908; transitional endoplasmic reticulum ATPase [KO:K13525 ]
131540115 zgc:73226; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 [KO:K15464 ]
131540516 si:dkey-71l1.1; mitochondrial import receptor subunit TOM40B isoform X1 [KO:K11518 ]
131540839 june; junE proto-oncogene, AP-1 transcription factor subunit [KO:K04448 ]
131542047 rhot1b; mitochondrial Rho GTPase 1b isoform X1 [KO:K07870 ] [EC:3.6.5.-]
131542824 tomm70a; LOW QUALITY PROTEIN: mitochondrial import receptor subunit TOM70 [KO:K17768 ]
131542936 atf4a; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374 ]
131546176 mon1ba; vacuolar fusion protein MON1 homolog B isoform X1 [KO:K20195 ]
131548951 map1lc3a; LOW QUALITY PROTEIN: microtubule-associated proteins 1A/1B light chain 3A [KO:K10435 ]
131549043 mon1bb; vacuolar fusion protein MON1 homolog B isoform X1 [KO:K20195 ]
131549357 tomm20b; mitochondrial import receptor subunit TOM20 homolog B isoform X1 [KO:K17770 ]
131549451 map1lc3c; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435 ]
131551186 bnip3; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 [KO:K15464 ]
131551575 calcoco2; LOW QUALITY PROTEIN: calcium-binding and coiled-coil domain-containing protein 2 [KO:K21348 ]
131552273 tomm20a; translocase of outer mitochondrial membrane 20 [KO:K17770 ]
131552302 hif1aa; hypoxia inducible factor 1 subunit alpha a isoform X1 [KO:K08268 ]
131553542 map1lc3cl; microtubule-associated proteins 1A/1B light chain 3C isoform X1 [KO:K10435 ]
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
