Deficiency in the membrane protein Tmbim3a/Grinaa initiates cold-induced ER stress and cell death by activating an intrinsic apoptotic pathway in zebrafish

J Biol Chem. 2019 Jul 26;294(30):11445-11457. doi: 10.1074/jbc.RA119.007813. Epub 2019 Jun 6.

Abstract

Most members of the family of proteins containing a transmembrane BAX inhibitor motif (TMBIM) have anti-apoptotic activity, but their in vivo functions and intracellular mechanisms remain obscure. Here, we report that zebrafish Tmbim3a/Grinaa functions in the prevention of cold-induced endoplasmic reticulum (ER) stress and apoptosis. Using a gene-trapping approach, we obtained a mutant zebrafish line in which the expression of the tmbim3a/grinaa gene is disrupted by a Tol2 transposon insertion. Homozygous tmbim3a/grinaa mutant larvae exhibited time-dependently increased mortality and apoptosis under cold exposure (at 16 °C). Mechanistically, using immunofluorescence, fluorescence-based assessments of intracellular/mitochondrial Ca2+ levels, mitochondrial membrane potential measurements, and Ca2+-ATPase assays, we found that cold exposure suppresses sarcoplasmic/ER Ca2+-ATPase (SERCA) activity and induces the unfolded protein response (UPR) and ER stress. We also found that the cold-induced ER stress is increased in homozygous tmbim3a/grinaa mutant embryos. The cold-stress hypersensitivity of the tmbim3a/grinaa mutants was tightly associated with disrupted intracellular Ca2+ homeostasis, followed by mitochondrial Ca2+ overload and cytochrome c release, leading to the activation of caspase 9- and caspase-3-mediated intrinsic apoptotic pathways. Treatment of zebrafish larvae with the intracellular Ca2+ chelator 1,2-bis(2-aminophenoxy)ethane-N,N,N',N'-tetraacetate-acetoxymethyl ester (BAPTA-AM) or with 2-aminoethoxydiphenyl borate (2-APB), an inhibitor of the calcium-releasing protein inositol 1,4,5-trisphosphate receptor (IP3R), alleviated cold-induced cell death. Together, these findings unveil a key role of Tmbim3a/Grinaa in relieving cold-induced ER stress and in protecting cells against caspase 9- and caspase 3-mediated apoptosis during zebrafish development.

Keywords: Grinaa; apoptosis; calcium homeostasis; calcium intracellular release; cell death; cold exposure; endoplasmic reticulum stress; endoplasmic reticulum stress (ER stress); stress response; transmembrane BAX inhibitor motif (TMBIM); unfolded protein response (UPR); zebrafish.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Apoptosis* / drug effects
  • Boron Compounds / pharmacology
  • Chelating Agents / pharmacology
  • Cold Temperature*
  • Egtazic Acid / analogs & derivatives
  • Egtazic Acid / pharmacology
  • Embryo, Nonmammalian / drug effects
  • Endoplasmic Reticulum Stress*
  • Membrane Proteins / genetics
  • Membrane Proteins / metabolism*
  • Mutation
  • Sarcoplasmic Reticulum Calcium-Transporting ATPases / metabolism
  • Subcellular Fractions / metabolism
  • Unfolded Protein Response
  • Zebrafish / embryology
  • Zebrafish / growth & development
  • Zebrafish Proteins / genetics
  • Zebrafish Proteins / metabolism*

Substances

  • Boron Compounds
  • Chelating Agents
  • Membrane Proteins
  • Zebrafish Proteins
  • 1,2-bis(2-aminophenoxy)ethane N,N,N',N'-tetraacetic acid acetoxymethyl ester
  • Egtazic Acid
  • 2-aminoethoxydiphenyl borate
  • Sarcoplasmic Reticulum Calcium-Transporting ATPases