OnCo

Protein homeostasis and the proteasome

Unwanted proteins are tagged with ubiquitin and fed into a shredder. Myeloma dies when the shredder jams; and the newest drugs forge the tags so that a cancer destroys its own oncoproteins.

Diagram

Pick a product above a diagram to see the nodes it hits and the escape routes below the block. Hover or tap any node or arrow for what it is; every node opens its target, glossary entry or the pathway page. Violet boxes are druggable targets.

Light up a product:+2 more via the Connected tab
E1 → E2 ubiquitin activates E3 ligase (CRBN, VHL, MDM2)E3 ligase (CRBN, VHL, MDM2) activates Substrate (IKZF1/3, p53, HIF)Substrate (IKZF1/3, p53, HIF) activates K48 ubiquitin chainDUBs (USP7) inhibits K48 ubiquitin chainK48 ubiquitin chain activates 26S proteasome (β5)26S proteasome (β5) activates Degradation26S proteasome (β5) inhibits UPR, IκB → NF-κBGlues / PROTACs hijack E3 activates E3 ligase (CRBN, VHL, MDM2)Glues / PROTACs hijack E3 activates Substrate (IKZF1/3, p53, HIF)HSP90 chaperones inhibits Substrate (IKZF1/3, p53, HIF)E1 → E2 ubiquitinE1 → E2 ubiquitinE3 ligase (CRBN, VHL, MDM2): MDM2 is the protein that degrades p53; blocking it reactivates p53 in tumours where the gene is intact, especially the liposarcomas that carry extra copies of MDM2.E3 ligase (CRBN, VHL, MDM…Substrate (IKZF1/3, p53, HIF): A master switch that kidney cancer cells leave permanently on when they lose the VHL gene; belzutifan blocks it.Substrate (IKZF1/3, p53, …K48 ubiquitin chainK48 ubiquitin chainDUBs (USP7)DUBs (USP7)26S proteasome (β5)26S proteasome (β5)UPR, IκB → NF-κBUPR, IκB → NF-κBGlues / PROTACs hijack E3: The hormone switch that drives most breast cancers. Blocking or destroying it is the oldest and most effective targeted therapy.Glues / PROTACs hijack E3DegradationDegradationHSP90 chaperonesHSP90 chaperonesactivatesinhibitsdruggable target (click)hit by selected productescape route
Ubiquitin-proteasome system & protein homeostasisCells tag unwanted proteins with a small marker called ubiquitin and feed them into a shredder, the proteasome. Myeloma cells, which make antibody in bulk, die if the shredder jams; and the newest drugs hijack the tagging machinery to make a cancer destroy its own oncoproteins.

A recycling plant with barcode stickers (ubiquitin) and a shredder (proteasome). Myeloma is a paper mill that produces so much waste it dies when the shredder stops (bortezomib). PROTACs and glues are forged stickers that get the plant to shred the cancer's own machinery.

What happens

In plain words, then the glossary entries the stage rests on. Chapter 3, Replication and growth machinery: Cancer cells use the same engine as normal cells, only stuck at full throttle.

Unwanted proteins are tagged with ubiquitin and fed into a shredder. Myeloma dies when the shredder jams; and the newest drugs forge the tags so that a cancer destroys its own oncoproteins.

Ubiquitin-proteasome system & protein homeostasis. Cells tag unwanted proteins with a small marker called ubiquitin and feed them into a shredder, the proteasome. Myeloma cells, which make antibody in bulk, die if the shredder jams; and the newest drugs hijack the tagging machinery to make a cancer destroy its own oncoproteins.

The molecular players

The proteins and genes at this stage, with their role and how many products act on each. Listed players come from the atlas; drawn players sit as nodes in the diagrams above.

Where medicines act

Products grouped by the node they hit, most advanced first, with the cancers an approved product is linked to. Pick one above the diagram to see it light up.

AtMDM2node E3 ligase (CRBN, VHL, MDM2) in Ubiquitin-proteasome system & protein homeostasis2 products
AtHIF-2αnode Substrate (IKZF1/3, p53, HIF) in Ubiquitin-proteasome system & protein homeostasis1 product

How tumours escape

Records tied to this stage that describe resistance, evasion or tolerance. The resistance atlas lists the routes class by class.

Measured by

Biomarkers, tests and assays in the corpus that read this stage in a patient.

Open questions

What is not known at this stage: the atlas's own questions, the bottlenecks it bears on, and the ideas in the corpus that try to answer them.

  • How many of the ~600 E3 ligases can be recruited by glues, and do tissue-restricted ligases give tumour selectivity?
  • Will degraders beat inhibitors on resistance, or just move it?

Key evidence

Papers in the corpus tied to this stage's pathways, targets and terms, newest first.

How this page is built: the stage is one entry in a curated atlas (src/data/mechanics-atlas.ts). Players, medicines, escape routes, tests, ideas and papers are resolved from the knowledge graph at build time through the stage's pathways, targets and terms, so every item here has its own page and sources. Where a section is missing, the corpus has no record tied to the stage yet. Nothing here is medical advice; see about and methodology. Stage 3.8 of 56.