Entry Name Mitophagy - animal - Macaca mulatta (rhesus 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 Ortholog table Other DBs Organism Macaca mulatta (rhesus monkey) [GN:
mcc ]
Gene 714434 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985 ]
706037 PINK1; serine/threonine-protein kinase PINK1, mitochondrial isoform X1 [KO:K05688 ] [EC:2.7.11.1 ]
705701 TOMM7; mitochondrial import receptor subunit TOM7 homolog [KO:K17771 ]
713297 TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518 ]
100426891 TOMM40L; mitochondrial import receptor subunit TOM40B [KO:K11518 ]
114675319 mitochondrial import receptor subunit TOM40B isoform X1 [KO:K11518 ]
700628 TOMM20L; TOMM20-like protein 1 isoform X2 [KO:K17770 ]
712039 TOMM20; mitochondrial import receptor subunit TOM20 homolog [KO:K17770 ]
700347 TOMM70; mitochondrial import receptor subunit TOM70 [KO:K17768 ]
717901 RHOT1; mitochondrial Rho GTPase 1 isoform X1 [KO:K07870 ] [EC:3.6.5.-]
722352 RHOT2; mitochondrial Rho GTPase 2 isoform X3 [KO:K07871 ] [EC:3.6.5.-]
720243 UBA52; ubiquitin-60S ribosomal protein L40 [KO:K02927 ]
712934 UBC; LOW QUALITY PROTEIN: polyubiquitin-C [KO:K08770 ]
697836 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2 isoform X1 [KO:K21348 ]
708558 NBR1; next to BRCA1 gene 1 protein isoform X1 [KO:K17987 ]
702827 gamma-aminobutyric acid receptor-associated protein [KO:K08341 ]
714413 GABARAPL2; gamma-aminobutyric acid receptor-associated protein-like 2 [KO:K08341 ]
717640 GABARAPL1; gamma-aminobutyric acid receptor-associated protein-like 1 [KO:K08341 ]
714597 GABARAP; gamma-aminobutyric acid receptor-associated protein [KO:K08341 ]
706487 MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435 ]
695419 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435 ]
707439 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435 ]
697790 microtubule-associated proteins 1A/1B light chain 3B [KO:K10435 ]
693782 PGAM5; serine/threonine-protein phosphatase PGAM5, mitochondrial isoform X1 [KO:K15637 ] [EC:3.1.3.16 ]
709370 FUNDC1; FUN14 domain-containing protein 1 [KO:K17986 ]
100426120 BNIP3; LOW QUALITY PROTEIN: BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 [KO:K15464 ]
114674310 BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 [KO:K15464 ]
641440 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like [KO:K15465 ]
709825 BCL2L13; bcl-2-like protein 13 isoform X4 [KO:K15485 ]
715675 SAMM50; sorting and assembly machinery component 50 homolog [KO:K07277 ]
699575 EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860 ] [EC:2.7.11.1 ]
710150 EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
703965 ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
715919 RELA; transcription factor p65 isoform X1 [KO:K04735 ]
704678 HIF1A; hypoxia-inducible factor 1-alpha isoform X4 [KO:K08268 ]
707729 CITED2; cbp/p300-interacting transactivator 2 [KO:K21361 ]
695537 MITF; microphthalmia-associated transcription factor [KO:K09455 ]
704497 MON1A; vacuolar fusion protein MON1 homolog A isoform X2 [KO:K20195 ]
715104 MON1B; vacuolar fusion protein MON1 homolog B isoform X2 [KO:K20195 ]
718128 CCZ1; vacuolar fusion protein CCZ1 homolog isoform X1 [KO:K27045 ]
718743 TBC1D15; TBC1 domain family member 15 isoform X1 [KO:K20168 ]
698707 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 LinkDB All DBs
