Related Experiment Video

Updated: May 25, 2025

Parallel Interrogation of β-Arrestin2 Recruitment for Ligand Screening on a GPCR-Wide Scale using PRESTO-Tango Assay
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Parallel Interrogation of β-Arrestin2 Recruitment for Ligand Screening on a GPCR-Wide Scale using PRESTO-Tango Assay

Published on: March 10, 2020

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TANGO2 is an acyl-CoA binding protein

Agustin Leonardo Lujan1, Ombretta Foresti1, Jose Wojnacki1

  • 1Centre for Genomic Regulation (CRG), The Barcelona Institute for Science and Technology , Barcelona, Spain.

The Journal of Cell Biology
|February 27, 2025
PubMed

Insights

TANGO2 protein loss causes metabolic crises. Our study shows TANGO2 binds acyl-CoA in mitochondria, and mutations disrupt this, offering new therapeutic targets for TANGO2-related diseases.

Area of Science:

  • Biochemistry
  • Cell Biology
  • Human Genetics

Background:

  • TANGO2 deficiency leads to metabolic crises during high energy demand.
  • TANGO2's roles in lipid metabolism and heme transport are known, but its cellular localization is unclear.

Purpose of the Study:

  • To determine the cellular localization of TANGO2.
  • To investigate the biochemical function of TANGO2.
  • To understand the molecular basis of TANGO2-related disorders.

Main Methods:

  • Immunofluorescence microscopy to determine TANGO2 localization.
  • Site-directed mutagenesis to study TANGO2 function.
  • Biochemical assays to assess acyl-coenzyme A binding.

Main Results:

  • TANGO2 localizes to the mitochondrial lumen, mediated by a region with LIL residues.
  • Mutations in LIL residues cause TANGO2 mislocalization to lipid droplets.
  • TANGO2 binds acyl-coenzyme A, and mutations in the NRDE sequence impair this binding.

Conclusions:

  • TANGO2 functions as an acyl-coenzyme A binding protein within mitochondria.
  • Understanding TANGO2's biochemical role may lead to treatments for associated cardiomyopathies and rhabdomyolysis.

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