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TitleFunctional and structural basis of a hypermorphic TRPC3 variant.
Journal, issue, pagesSci Adv, Vol. 12, Issue 13, Page eaec9284, Year 2026
Publish dateMar 27, 2026
AuthorsBriar Bell / Angela M Jaramillo-Granada / Luis O Romero / Irene A Gutierrez / Venkata K P S Mallampalli / Guizhen Fan / Sameer Varma / Matthew L Baker / Irina I Serysheva / Valeria Vásquez / Julio F Cordero-Morales /
PubMed AbstractCerebellar ataxias are characterized by impaired motor coordination resulting from neuronal dysfunction within the cerebellum. The mechanisms underlying this pathology and its cerebellar-specific ...Cerebellar ataxias are characterized by impaired motor coordination resulting from neuronal dysfunction within the cerebellum. The mechanisms underlying this pathology and its cerebellar-specific neurodegeneration remain unknown. We uncover how a gain-of-function canonical transient receptor potential member 3 (TRPC3) mutation, coupled with a cerebellum-specific isoform, stabilizes the channel's open state, resists the leading inhibitor Pyr3, and drives calcium-dependent cell death. Restoring calcium homeostasis by expressing a Purkinje cell calcium pump improves cell viability. Transgenic expression of the TRPC3 hypermorphic variant in induces neurodegeneration, confirming its pathogenicity across species. Cryo-electron microscopy and molecular simulations reveal the structural basis for the stabilization of the cerebellar-specific TRPC3 variant in its open state and uncover a druggable allosteric inhibitory binding site. These findings provide an explanation for the vulnerability of cerebellar neurons in TRPC3-associated ataxias and highlight a site for therapeutic intervention.
External linksSci Adv / PubMed:41880503 / PubMed Central
MethodsEM (single particle)
Resolution2.5 - 3.3 Å
Structure data

70595

9olk

EMDB-70595, PDB-9olk:
Structure of wild-type human TRPC3
Method: EM (single particle) / Resolution: 2.8 Å

70596

9oll

EMDB-70596, PDB-9oll:
Structure of human TRPC3 T573A mutant
Method: EM (single particle) / Resolution: 3.1 Å

70597

9olm

EMDB-70597, PDB-9olm:
Structure of human TRPC3 cerebellar splice variant (isoform c)
Method: EM (single particle) / Resolution: 2.5 Å

70601

9olx

EMDB-70601, PDB-9olx:
Structure of a constitutively open human TRPC3 mutant in the inhibited state
Method: EM (single particle) / Resolution: 3.3 Å

70724

9opu

EMDB-70724, PDB-9opu:
Structure of a constitutively open human TRPC3 mutant
Method: EM (single particle) / Resolution: 3.3 Å

Chemicals

ChemComp-CPL:
1-PALMITOYL-2-LINOLEOYL-SN-GLYCERO-3-PHOSPHOCHOLINE / phospholipid*YM


ChemComp, No image

ChemComp-UNL:
Unknown ligand

PDB-1ccx:
Unknown entry

Source
  • homo sapiens (human)
KeywordsMEMBRANE PROTEIN / TRPC3 / cerebellar ataxia / moonwalker

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