Structural basis of purine nucleotide inhibition of human uncoupling protein 1

Sci Adv. 2023 Jun 2;9(22):eadh4251. doi: 10.1126/sciadv.adh4251. Epub 2023 May 31.

Abstract

Mitochondrial uncoupling protein 1 (UCP1) gives brown adipose tissue of mammals its specialized ability to burn calories as heat for thermoregulation. When activated by fatty acids, UCP1 catalyzes the leak of protons across the mitochondrial inner membrane, short-circuiting the mitochondrion to generate heat, bypassing ATP synthesis. In contrast, purine nucleotides bind and inhibit UCP1, regulating proton leak by a molecular mechanism that is unclear. We present the cryo-electron microscopy structure of the GTP-inhibited state of UCP1, which is consistent with its nonconducting state. The purine nucleotide cross-links the transmembrane helices of UCP1 with an extensive interaction network. Our results provide a structural basis for understanding the specificity and pH dependency of the regulatory mechanism. UCP1 has retained all of the key functional and structural features required for a mitochondrial carrier-like transport mechanism. The analysis shows that inhibitor binding prevents the conformational changes that UCP1 uses to facilitate proton leak.

MeSH terms

  • Cryoelectron Microscopy
  • Humans
  • Ion Channels* / chemistry
  • Mitochondrial Proteins / metabolism
  • Protons*
  • Purine Nucleotides
  • Uncoupling Protein 1 / genetics
  • Uncoupling Protein 1 / metabolism

Substances

  • Ion Channels
  • Mitochondrial Proteins
  • Protons
  • Purine Nucleotides
  • Uncoupling Protein 1
  • UCP1 protein, human