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Chaperone mediated autophagy (CMA) is a selective autophagy pathway whereby cytosolic proteins bearing a recognition motif are individually recognized and translocated into the lysosomal lumen for degradation. The KFERQ motif s recognized by the Hsc70 chaperone Hspa8, a chaperone/co-chaperone complex targets the substrate to the lysosomal membrane where it interacts with the cytosolic tail of single-span membrane protein Lamp2a. Lamp2a multimerization and formation of a 700kDa complex promotes

Pathway Diagram:

Elsevier Inc. CMA targets cathepsins LysHspa8 Lamp2a multimer Bag1 ---> ATP ADP ---> ATP Hsp8a factors ADP-bound Hspa8 state ATP-bound Hspa8 state Gfap dissoc ---- P-Gfap Eef1a1 Gfap Lamp2a dissoc KFERQ motif ---- Lamp2 Eef1a1 ---- P-Gfap Bag1 Hspa8 ---- KFERQ motif Gfap ---- Lamp2a multimer Dnajb1 Hsp8a factors ---- Hspa8 ATP ---- Hspa8 ATP ADP ---- Hspa8 ADP Dnajb1 ---> ADP ATP ---> ADP Hspa8 Lamp2 KFERQ motif ---> KFERQ translocated CMA target degradation cathepsins ---> CMA targets KFERQ motif KFERQ translocated Gfap dissoc P-Gfap

Genes in Pathway:

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chaperone mediated autophagy pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Bag1 Bcl2 associated athanogene 1 JBrowse link 5 57,254,421 57,267,002 RGD:10755685
G Ctsb cathepsin B JBrowse link 15 46,316,741 46,337,613 RGD:10755730
G Ctsd cathepsin D JBrowse link 1 215,541,570 215,553,446 RGD:10755729
G Ctsl cathepsin L JBrowse link 17 1,873,105 1,879,266 RGD:10755730
G Dnajb1 DnaJ heat shock protein family (Hsp40) member B1 JBrowse link 19 24,747,178 24,750,919 RGD:10755685
G Eef1a1 eukaryotic translation elongation factor 1 alpha 1 JBrowse link 8 85,838,594 85,841,816 RGD:10755322
G Gfap glial fibrillary acidic protein JBrowse link 10 90,990,762 90,999,435 RGD:10755322
G Hsp90aa1 heat shock protein 90 alpha family class A member 1 JBrowse link 6 135,107,262 135,112,793 RGD:10755685
G Hspa8 heat shock protein family A (Hsp70) member 8 JBrowse link 8 44,989,401 44,993,261 RGD:10755322
G Lamp2 lysosomal-associated membrane protein 2 JBrowse link X 124,722,628 124,766,079 RGD:10755322
G St13 ST13, Hsp70 interacting protein JBrowse link 7 122,585,770 122,635,731 RGD:10755685
G Stip1 stress-induced phosphoprotein 1 JBrowse link 1 222,274,133 222,293,139 RGD:10755685

Pathway Gene Annotations

Disease Annotations Associated with Genes in the chaperone mediated autophagy pathway
Disease TermsGene Symbols
Acute Coronary SyndromeHspa8
acute kidney failureHspa8
Acute Liver FailureHspa8
Acute Necrotizing PancreatitisCtsb
AlbuminuriaCtsb , Ctsl
Alcoholic Fatty LiverCtsb
Alexander DiseaseGfap
Alzheimer's diseaseCtsd , Hsp90aa1
amyotrophic lateral sclerosisGfap
amyotrophic lateral sclerosis type 1Ctsd , Gfap
anaplastic large cell lymphomaHsp90aa1
Animal Disease ModelsGfap
Animal Mammary NeoplasmsEef1a1
atherosclerosisCtsb , Ctsd , Ctsl
autistic disorderLamp2
brain edemaGfap
brain infarctionCtsb
Brain Injuries, TraumaticLamp2
brain ischemiaCtsb , Ctsl , Hspa8
breast cancerBag1 , Ctsb , Ctsd
Breast NeoplasmsBag1 , Hsp90aa1
cervical cancerHspa8
cervix carcinomaBag1
Chemical and Drug Induced Liver InjuryHspa8
chronic conjunctivitisLamp2
Ciliary Motility DisordersGfap
cognitive disorderLamp2
Cognitive DysfunctionHsp90aa1
colitisCtsb , Ctsl
colorectal cancerHspa8
contact dermatitisHsp90aa1
Danon diseaseLamp2
diabetes mellitusCtsb
Diabetes Mellitus, Experimental Ctsb , Ctsl , Hsp90aa1
Diabetic NephropathiesHspa8
dilated cardiomyopathyCtsl , Lamp2
disease of metabolismHspa8
Disease ProgressionCtsb
Ductal CarcinomaHsp90aa1
endometrial cancerBag1
esophagus adenocarcinomaCtsb
Experimental ArthritisCtsb
Experimental Autoimmune Myasthenia GravisCtsl
familial hypertrophic cardiomyopathyLamp2
fatty liver diseaseCtsb
Frontotemporal Lobar DegenerationHsp90aa1
genetic diseaseGfap
glioblastoma multiformeCtsb
Graft vs Host DiseaseHspa8
Guillain-Barre syndromeCtsb
Head Injuries, ClosedGfap
Head Injuries, PenetratingGfap
hepatocellular carcinomaCtsb , Ctsd , Ctsl
human immunodeficiency virus infectious diseaseHsp90aa1
Huntington's diseaseBag1 , Hspa8
hypertrophic cardiomyopathyCtsl , Lamp2
inflammatory bowel diseaseHspa8
Intervertebral Disc DisplacementCtsl
intracranial aneurysmCtsb
invasive ductal carcinomaBag1
Keratolytic Winter ErythemaCtsb
kidney diseaseCtsd
Kidney Reperfusion InjuryHspa8
Knee OsteoarthritisEef1a1 , Hsp90aa1
lesion of sciatic nerveBag1
Lewy body dementiaGfap
Lymphatic MetastasisBag1
macular degenerationHspa8
malignant gliomaCtsb
Mammary Neoplasms, ExperimentalEef1a1
Mercury Poisoning, Nervous SystemCtsb
Mouth NeoplasmsHspa8
multiple sclerosisCtsb , Ctsl , Hspa8
muscular atrophyCtsl
muscular diseaseCtsb
myocardial infarctionCtsb , Lamp2
Neoplasm InvasivenessCtsb , Ctsd
Neoplasm MetastasisCtsb , Ctsd , Hsp90aa1
nephrotic syndromeCtsl
nervous system diseaseGfap
neuromyelitis opticaGfap
neuronal ceroid lipofuscinosisCtsd
neuronal ceroid lipofuscinosis 1Gfap
neuronal ceroid lipofuscinosis 10Ctsd
Ocular Paraneoplastic SyndromesHspa8
Optic Nerve InjuriesCtsb
otitis mediaCtsb , Ctsl
Pancreatitis, Experimental, Acute Lamp2
Parkinson's diseaseGfap , Hspa8
peripheral nervous system diseaseGfap
polycystic kidney diseaseCtsb
portal hypertensionHsp90aa1
primary ciliary dyskinesiaGfap
progressive myoclonus epilepsyCtsd
prostate cancerBag1
Prostatic NeoplasmsCtsb , Ctsd
proteinuriaCtsb , Ctsl
relapsing-remitting multiple sclerosisHspa8
renal cell carcinomaCtsb , Ctsd
renal hypertensionHspa8
renal tubular acidosisCtsb
Reoviridae InfectionsCtsb , Ctsl
Reperfusion InjuryBag1 , Ctsb , Hspa8
retinal diseaseGfap
rheumatoid arthritisCtsd , Hsp90aa1
sciatic neuropathyLamp2
Spina Bifida CysticaGfap
Spinal Cord InjuriesGfap
spinocerebellar ataxia type 17Hspa8
Sprains and StrainsCtsl
squamous cell carcinomaHspa8
status epilepticusLamp2
Stomach NeoplasmsCtsl , Eef1a1 , Hspa8
transient cerebral ischemiaHspa8
transitional cell carcinomaCtsb
Trifascicular BlockLamp2
trigeminal neuralgiaGfap
type 1 diabetes mellitusCtsb , Ctsl
type 2 diabetes mellitusCtsb , Ctsl
urinary bladder cancerCtsb
Urination DisordersGfap
visual epilepsyCtsd
Weight GainCtsd
Pathway Annotations Associated with Genes in the chaperone mediated autophagy pathway
Phenotype Annotations Associated with Genes in the chaperone mediated autophagy pathway

References Associated with the chaperone mediated autophagy pathway:

Ontology Path Diagram:

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