Retinoic acid receptor alpha (RARA)
The retinoic acid receptor that, fused to PML in acute promyelocytic leukaemia, blocks white blood cells from maturing. All-trans retinoic acid releases the block and arsenic trioxide destroys the fusion protein, turning a once rapidly fatal leukaemia into one of the most curable.
Overview
RARA is a nuclear receptor that switches on genes of myeloid differentiation when bound by retinoic acid. In acute promyelocytic leukaemia the t(15;17) translocation fuses PML to RARA, and the fusion protein recruits co-repressors that lock promyelocytes in an immature state. Pharmacological doses of all-trans retinoic acid (tretinoin) release the co-repressors and force differentiation, while arsenic trioxide binds the PML moiety and triggers degradation of the fusion protein. Given together, without chemotherapy in standard-risk disease, they cure most patients; differentiation syndrome is the characteristic early complication.
- Target · the protein and the cell it sits on
- Drug · antibody, small molecule, cell or radioligand
- Effect · signal, damage or kill
In plain words · The retinoic acid receptor that, fused to PML in acute promyelocytic leukaemia, blocks white blood cells from maturing. All-trans retinoic acid releases the block and arsenic trioxide destroys the fusion protein, turning a once rapidly fatal leukaemia into one of the most curable.
- 1 · What it is
The retinoic acid receptor that, fused to PML in acute promyelocytic leukaemia, blocks white blood cells from maturing. All-trans retinoic acid releases the block and arsenic trioxide destroys the fusion protein, turning a once rapidly fatal leukaemia into one of the most curable.
- 2 · What goes wrong in cancer
Ligand-activated nuclear receptor of myeloid differentiation; the PML::RARA fusion in APL is the drug target of tretinoin and arsenic trioxide.
- 3 · How drugs use it
No product in this corpus aims at Retinoic acid receptor alpha (RARA) yet. Drugs cut off the hormone supply, block the receptor so the hormone cannot bind, or send the receptor to the cell's waste disposal.
External identifiers
Biology
Ligand-activated nuclear receptor of myeloid differentiation; the PML::RARA fusion in APL is the drug target of tretinoin and arsenic trioxide.
- Acute promyelocytic leukaemia (PML::RARA fusion in almost all cases)
Latest papers
topQuery for this target: (TITLE:"Retinoic acid receptor alpha" OR ABSTRACT:"Retinoic acid receptor alpha" OR TITLE:"RARA" OR ABSTRACT:"RARA") AND (cancer OR tumor OR tumour OR oncology OR carcinoma OR lymphoma OR leukemia OR leukaemia OR myeloma OR sarcoma OR melanoma OR glioma). Results are unfiltered search hits about Retinoic acid receptor alpha (RARA), not a curated reading list.
Similar pages
not linked directly; found by shared links- PersonSaijuan Chen
Shares Tretinoin (all-trans retinoic acid, ATRA), Arsenic trioxide, Acute myeloid leukaemia.
- PersonZhu Chen
Shares Tretinoin (all-trans retinoic acid, ATRA), Arsenic trioxide, Acute myeloid leukaemia.
- InstitutionRuijin Hospital, Shanghai Jiao Tong University
Shares Tretinoin (all-trans retinoic acid, ATRA), Arsenic trioxide, Acute myeloid leukaemia.
- TermDifferentiation syndrome
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- PersonJanet Rowley
Shares Tretinoin (all-trans retinoic acid, ATRA), Acute myeloid leukaemia.
- PathwayAcute myeloid leukaemia (KEGG map)
Shares Arsenic trioxide, Acute myeloid leukaemia.