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The double membrane-bound mitochondria organelles play essential roles in cellular homeostasis. In addition to the major function of ATP production in the oxidative phosphorylation (OXPHOS) pathway, mitochondria are the site of other important metabolic pathway such as fatty acid oxidation, citric acid cycle and urea cycle, heme and iron-sulfur cluster biosynthesis. They also provide for calcium storage and signaling, and have a central role in apoptosis. The mitochondrial distribution/mass var

Pathway Diagram:

Elsevier Inc. Fundc1 Atxn3 Sqstm1 canonical K48, K63 and noncanonical K6, K11 linkages deubiquitinases other autophagy/mitophagy components Map1lc3a Map1lc3b Gabarap Optn translocated cardiolipin Pink1_IMM Parkin substrates ---| mitochondria transport pathway Parkin substrates Park2 ---| mitochondria transport pathway Park2 Parkin substrates ---| mitochondria fusion pathway Map1lc3b ---- translocated cardiolipin casein kinase 2 autophagophore recruitment Pgam5 Bnip3l ---- Map1lc3b Bnip3l ---- Gabarap canonical K48, K63 and noncanonical K6, K11 linkages ---- Optn canonical K48, K63 and noncanonical K6, K11 linkages ---- Sqstm1 Src ---| Fundc1 Src ---> Fundc1 Pgam5 ---> Fundc1 Pgam5 --+> Fundc1 Fundc1 ---- Map1lc3b Bnip3 ---- Map1lc3b Fundc1 ---- Map1lc3a Bnip3l ---- Map1lc3a Map1lc3b ---- Optn Map1lc3b ---- Sqstm1 Map1lc3a ---- Optn Map1lc3a ---- Sqstm1 casein kinase 2 ---| Fundc1 casein kinase 2 ---> Fundc1 Src Pink1 ---> Pink1_IMM Parl ---| Pink1_IMM Parl Parl ---> Pink1_IMM Pink1 Tomm7 Tomm7 --+> Pink1 deubiquitinases ---> Parkin substrates Pink1 ---> Park2 Pink1 --+> Park2 Bnip3 ---- Map1lc3a Atxn3 ---| Park2 Ube2l3 Atxn3 ---- Ube2l3 mitochondria transport pathway mitochondria fusion pathway cardiolipin metabolic pathway Bnip3 hypoxia inducible factor pathway Bnip3l hypoxia inducible factor pathway ---> Bnip3l hypoxia inducible factor pathway ---> Bnip3 Park2 ---> canonical K48, K63 and noncanonical K6, K11 linkages Park2 ---> Parkin substrates

Genes in Pathway:

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mitochondrial autophagy pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Ambra1 autophagy and beclin 1 regulator 1 JBrowse link 3 80,634,470 80,830,068 RGD:10401098
G Atg7 autophagy related 7 JBrowse link 4 146,570,113 146,777,093 RGD:10400888
G Atxn3 ataxin 3 JBrowse link 6 125,817,420 125,853,461 RGD:10401790
G Becn1 beclin 1 JBrowse link 10 89,209,944 89,225,297 RGD:10401098
G Bnip3 BCL2 interacting protein 3 JBrowse link 1 211,248,098 211,265,282 RGD:10400888
G Bnip3l BCL2 interacting protein 3 like JBrowse link 15 43,643,897 43,667,123 RGD:10400888
G Csnk2a1 casein kinase 2 alpha 1 JBrowse link 3 147,713,808 147,760,375 RGD:10400888
G Csnk2a2 casein kinase 2 alpha 2 JBrowse link 19 9,972,537 10,012,043 RGD:10400888
G Csnk2b casein kinase 2 beta JBrowse link 20 5,108,692 5,113,675 RGD:10400888
G Fundc1 FUN14 domain containing 1 JBrowse link X 5,549,468 5,566,125 RGD:10400888
G Optn optineurin JBrowse link 17 77,167,700 77,218,374 RGD:10401790
G Parl presenilin associated, rhomboid-like JBrowse link 11 84,517,368 84,544,463 RGD:10402124
G Pgam5 PGAM family member 5, mitochondrial serine/threonine protein phosphatase JBrowse link 12 52,467,417 52,474,533 RGD:10400888
G Pink1 PTEN induced putative kinase 1 JBrowse link 5 156,677,146 156,689,258 RGD:10400888
G Prkn parkin RBR E3 ubiquitin protein ligase JBrowse link 1 48,880,015 50,069,998 RGD:10401098
G Sqstm1 sequestosome 1 JBrowse link 10 35,704,728 35,716,316 RGD:10401098
G Src SRC proto-oncogene, non-receptor tyrosine kinase JBrowse link 3 153,547,807 153,595,643 RGD:10400888
G Tomm7 translocase of outer mitochondrial membrane 7 JBrowse link 4 7,835,949 7,842,790 RGD:10402124
G Ube2l3 ubiquitin-conjugating enzyme E2L 3 JBrowse link 11 88,047,186 88,088,477 RGD:10401790
G Ulk1 unc-51 like autophagy activating kinase 1 JBrowse link 12 51,908,105 51,934,704 RGD:10400888
G Usp15 ubiquitin specific peptidase 15 JBrowse link 7 66,595,707 66,687,707 RGD:10401790
G Usp30 ubiquitin specific peptidase 30 JBrowse link 12 48,262,663 48,316,580 RGD:10401790
G Usp8 ubiquitin specific peptidase 8 JBrowse link 3 119,173,818 119,222,499 RGD:10401790
altered mitochondrial autophagy pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Pink1 PTEN induced putative kinase 1 JBrowse link 5 156,677,146 156,689,258 RGD:10450527
G Prkn parkin RBR E3 ubiquitin protein ligase JBrowse link 1 48,880,015 50,069,998 RGD:10450527

Pathway Gene Annotations

Disease Annotations Associated with Genes in the mitochondrial autophagy pathway
Disease TermsGene Symbols
ACTH-Secreting Pituitary AdenomaUsp8
Adenocarcinoma of EsophagusBecn1
Adenocarcinoma of LungPrkn
Alzheimer DiseaseBecn1 , Prkn
Amyotrophic Lateral SclerosisBecn1 , Optn , Sqstm1
Amyotrophic Lateral Sclerosis 12Optn
Amyotrophic Lateral Sclerosis, Autosomal RecessiveOptn
Autistic DisorderPrkn
Barrett EsophagusBecn1
Brain InfarctionBecn1
Brain InjuriesBecn1
Brain IschemiaBecn1 , Src
Breast NeoplasmsCsnk2a1 , Parl , Src
Carcinoma, HepatocellularPrkn
Carcinoma, Intraductal, NoninfiltratingBnip3 , Src
Carcinoma, Squamous CellCsnk2a1 , Csnk2a2 , Csnk2b
CardiomegalyBecn1 , Src
Cardiomyopathy, HypertrophicBecn1
Cerebral HemorrhageBecn1 , Bnip3l , Sqstm1
Chronic Cerebral HypoperfusionBnip3l
Chronic Intermittent HypoxiaBecn1 , Bnip3
Cognition DisordersPrkn
Colonic NeoplasmsBecn1 , Prkn , Src
Colorectal NeoplasmsSrc
Congenital Disorders of GlycosylationPink1
Coronary Artery DiseaseParl
Currarino TriadParl
Dental Pulp ExposureOptn
Depressive DisorderBecn1
Diabetes Mellitus, ExperimentalAtg7 , Becn1
Diabetes Mellitus, Type 2Becn1 , Parl , Prkn , Src
Diabetes, GestationalBnip3l
Diabetic NephropathiesBecn1
Diabetic NeuropathiesBecn1
Disease Models, AnimalPrkn
Encephalitis, ViralBecn1
Enterocolitis, NecrotizingBecn1 , Bnip3
Exfoliation SyndromeOptn
Experimental SeizuresBecn1
Familial Paget's Disease of BoneOptn , Sqstm1
Frontotemporal DementiaPrkn
Frontotemporal Dementia and/or Amyotrophic Lateral Sclerosis 3Sqstm1
Frontotemporal Lobar DegenerationOptn
GlaucomaBecn1 , Optn
Glaucoma 1, Open Angle, EOptn
Glaucoma, Normal Tension, Susceptibility ToOptn
Glaucoma, Open-AngleOptn
Glaucoma, Primary Open AngleOptn
GlioblastomaBecn1 , Prkn
Head Injuries, ClosedBecn1
Heart FailureBecn1 , Bnip3
Heat StrokeBecn1
Hepatitis B, ChronicUbe2l3
Huntington DiseaseOptn , Prkn
HypertensionAtg7 , Becn1 , Sqstm1 , Src
Hypertrophy, Left VentricularPrkn
Hypoglossal Nerve InjuriesCsnk2b
Hypoxia-Ischemia, BrainBecn1
Infarction, Middle Cerebral ArteryBecn1 , Bnip3 , Bnip3l
Insulin ResistanceParl
Intervertebral Disc DegenerationBecn1
Ischemic Attack, TransientBecn1 , Bnip3 , Optn , Parl , Prkn
Kabuki Syndrome 2Fundc1
Kidney Failure, ChronicBecn1 , Bnip3
Kidney Reperfusion InjuryBecn1 , Bnip3 , Src
Learning DisordersPrkn
Leukemia-Lymphoma, Adult T-CellCsnk2a1 , Csnk2b
Lewy Body DiseaseBecn1 , Prkn
Liver CirrhosisBecn1
Liver Cirrhosis, ExperimentalPrkn
Low Tension GlaucomaOptn
Lung NeoplasmsBecn1 , Prkn
Machado-Joseph DiseaseAtxn3 , Becn1
Macular DegenerationSqstm1
Mammary Neoplasms, ExperimentalCsnk2a1
Manganese PoisoningBnip3 , Prkn
Memory DisordersPrkn
Motor Neuron DiseaseOptn
Multiple MyelomaBnip3
Multiple SclerosisPrkn
Myocardial InfarctionAtg7 , Becn1 , Bnip3 , Csnk2a1 , Csnk2b
Myocardial Reperfusion InjuryBecn1 , Bnip3
Myoclonic Epilepsies, ProgressiveOptn
Neoplasm InvasivenessSrc
Neoplasm MetastasisSrc
Nerve DegenerationAtxn3 , Becn1 , Pink1 , Prkn
Neurodegenerative DiseasesAtg7
Neuronal Intranuclear Inclusion DiseaseOptn
Nonaka MyopathySqstm1
Optic Atrophy, Hereditary, LeberParl
Optic Neuropathy, IschemicBecn1
Osteitis DeformansOptn , Sqstm1
Osteoarthritis, ExperimentalBecn1 , Ulk1
ovarian cancerPrkn
Ovarian CystsPrkn
Ovarian NeoplasmsAtg7 , Prkn
Oxygen-Induced RetinopathyCsnk2a1 , Csnk2a2 , Csnk2b
Pancreatitis, Acute NecrotizingBecn1
Pancreatitis, Experimental, Acute Becn1
ParaplegiaAtg7 , Becn1
Parkinson DiseaseOptn , Pink1 , Prkn
Parkinson Disease 6, Autosomal Recessive Early-OnsetPink1
Parkinson Disease, Late-OnsetPink1
Parkinson Disease, SecondaryPrkn
Parkinsonian DisordersBecn1 , Pink1 , Prkn , Src
Pituitary ACTH HypersecretionUsp8
Pleomorphic Xanthoastrocytoma Tomm7
Pneumonia, StaphylococcalPrkn
Polycystic Kidney DiseasesBecn1
Polycystic Kidney, Autosomal RecessiveSrc
Prostatic NeoplasmsBnip3 , Src
Pulmonary Disease, Chronic ObstructiveBnip3
Reperfusion InjuryBecn1 , Src
Retinal DegenerationOptn
Retinal DetachmentBnip3
SchizophreniaBecn1 , Prkn
Skin NeoplasmsCsnk2a1 , Csnk2a2 , Csnk2b
Smith-Magenis SyndromeBecn1
Spinal Cord InjuriesAtg7 , Becn1 , Bnip3 , Bnip3l , Ulk1
Spinal Cord Reperfusion InjuryBecn1
Spinocerebellar AtaxiasOptn
Status EpilepticusBecn1 , Src
Stomach NeoplasmsBnip3
Subarachnoid HemorrhageBecn1 , Bnip3 , Prkn
Temporomandibular Joint DisordersBecn1
Ureteral ObstructionBecn1
Urinary Bladder Neck ObstructionOptn
Urinary Bladder NeoplasmsSrc
Uterine Cervical NeoplasmsPrkn
Weight LossAtg7
Wounds and InjuriesOptn
Pathway Annotations Associated with Genes in the mitochondrial autophagy pathway
Pathway TermsGene Symbols
altered mitochondrial autophagy pathwayPink1 , Prkn
altered Reelin signaling pathwaySrc
altered ubiquitin/proteasome degradation pathwayPrkn
autophagy pathwayAtg7 , Becn1 , Ulk1
beta-barrel pathway of mitochondrial protein importTomm7
carrier pathway of mitochondrial protein import.Tomm7
dopamine signaling pathwaySrc
E-cadherin signaling pathwayCsnk2a1 , Csnk2a2 , Csnk2b , Src
eicosanoid signaling pathwaySrc
endocytosis pathwaySrc , Usp8
Endoplasmic Reticulum-associated degradation pathwayAtxn3 , Prkn
endothelin signaling pathwaySrc
ephrin - ephrin receptor bidirectional signaling axisSrc
epidermal growth factor/neuregulin signaling pathwaySrc
estrogen signaling pathwaySrc
FasL mediated signaling pathwaySrc
fibroblast growth factor signaling pathwaySrc
glypican signaling pathwaySrc
gonadotropin-releasing hormone signaling pathwaySrc
hypoxia inducible factor pathwayBnip3
inositol metabolic pathwayAmbra1 , Becn1
interleukin-1 signaling pathwaySqstm1
intrinsic apoptotic pathwayBnip3 , Bnip3l
measles infection pathwayCsnk2a1 , Csnk2a2 , Csnk2b
mitochondria dynamics pathwayPink1
mitochondrial autophagy pathwayAmbra1 , Atg7 , Atxn3 , Becn1 , Bnip3 , Bnip3l , Csnk2a1 , Csnk2a2 , Csnk2b , Fundc1 , Optn , Parl , Pgam5 , Pink1 , Prkn , Sqstm1 , Src , Tomm7 , Ube2l3 , Ulk1 , Usp15 , Usp30 , Usp8
mTOR signaling pathwayBnip3 , Ulk1
Mycobacterium tuberculosis infection pathwaySrc
nerve growth factor signaling pathwaySrc
nuclear factor kappa B signaling pathwayCsnk2a1 , Csnk2a2 , Csnk2b , Src
nuclear factor, erythroid 2 like 2 signaling pathwayPgam5 , Sqstm1
Parkinson disease pathwayPink1 , Prkn , Ube2l3
phosphatidylinositol 3-kinase class I signaling pathwaySrc
phosphatidylinositol 3-kinase-Akt signaling pathwaySrc
phosphoinositide metabolic pathwayAmbra1 , Becn1
platelet-derived growth factor signaling pathwayCsnk2a1 , Src
presequence pathway of mitochondrial protein importTomm7
Reelin signaling pathwaySrc
ribosome biogenesis pathwayCsnk2a1 , Csnk2a2 , Csnk2b
scatter factor/hepatocyte growth factor signaling pathwaySrc
signaling pathway pertinent to the brain and nervous systemSrc
somatostatin signaling pathwaySrc
sphingosine 1-phosphate signaling pathwaySrc
syndecan signaling pathwaySrc
tumor necrosis factor mediated signaling pathwaySqstm1
ubiquitin/proteasome degradation pathwayPrkn , Src , Ube2l3
vascular endothelial growth factor signaling pathwaySrc
Wnt signaling pathwayCsnk2a1 , Csnk2a2 , Csnk2b
Phenotype Annotations Associated with Genes in the mitochondrial autophagy pathway

References Associated with the mitochondrial autophagy pathway:

Ontology Path Diagram:

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Import into Pathway Studio: