Molecular mechanisms, and selective pharmacological rescue, of Rem-inhibited CaV1.2 channels in heart

Circ Res. 2010 Sep 3;107(5):620-30. doi: 10.1161/CIRCRESAHA.110.224717. Epub 2010 Jul 8.

Abstract

Rationale: In heart, Ca(2+) entering myocytes via Ca(V)1.2 channels controls essential functions, including excitation-contraction coupling, action potential duration, and gene expression. RGK GTPases (Rad/Rem/Rem2/Gem/Kir sub-family of Ras-like GTPases) potently inhibit Ca(V)1.2 channels, an effect that may figure prominently in cardiac Ca(2+) homeostasis under physiological and disease conditions.

Objective: To define the mechanisms and molecular determinants underlying Rem GTPase inhibition of Ca(V)1.2 channels in heart and to determine whether such inhibited channels can be pharmacologically rescued.

Methods and results: Overexpressing Rem in adult guinea pig heart cells dramatically depresses L-type calcium current (I(Ca,L)) ( approximately 90% inhibition) and moderately reduces maximum gating charge (Q(max)) (33%), without appreciably diminishing the physical number of channels in the membrane. Rem-inhibited Ca(V)1.2 channels were supramodulated by BAY K 8644 (10-fold increase) compared to control channels (3-fold increase). However, Rem prevented protein kinase A-mediated upregulation of I(Ca,L), an effect achieved without disrupting the sympathetic signaling cascade because protein kinase A modulation of I(KS) (slow component of the delayed rectifier potassium current) remained intact. In accord with its functional impact on I(Ca,L), Rem selectively prevented protein kinase A- but not BAY K 8644-induced prolongation of the cardiac action potential duration. A GTP-binding-deficient Rem[T94N] mutant was functionally inert with respect to I(Ca,L) inhibition. A chimeric construct, Rem(265)-H, featuring a swap of the Rem C-terminal tail for the analogous domain from H-Ras, inhibited I(Ca,L) and Q(max) to the same extent as wild-type Rem, despite lacking the capacity to autonomously localize to the sarcolemma.

Conclusions: Rem predominantly inhibits I(Ca,L) in heart by arresting surface Ca(V)1.2 channels in a low open probability gating mode, rather than by interfering with channel trafficking. Moreover, Rem-inhibited Ca(V)1.2 channels can be selectively rescued by BAY K 8644 but not protein kinase A-dependent phosphorylation. Contrary to findings in reconstituted systems, Rem-induced ablation of cardiac I(Ca,L) requires GTP-binding, but not membrane-targeting of the nucleotide binding domain. These findings provide a different perspective on the molecular mechanisms and structural determinants underlying RGK GTPase inhibition of Ca(V)1.2 channels in heart, and suggest new (patho)physiological dimensions of this crosstalk.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • 3-Pyridinecarboxylic acid, 1,4-dihydro-2,6-dimethyl-5-nitro-4-(2-(trifluoromethyl)phenyl)-, Methyl ester / pharmacology
  • Action Potentials
  • Amino Acid Sequence
  • Animals
  • Binding Sites
  • Calcium Channel Agonists / pharmacology
  • Calcium Channels, L-Type / drug effects
  • Calcium Channels, L-Type / metabolism*
  • Calcium Signaling* / drug effects
  • Cells, Cultured
  • Cyclic AMP-Dependent Protein Kinases / metabolism
  • Guanosine Triphosphate / metabolism
  • Guinea Pigs
  • Ion Channel Gating* / drug effects
  • Male
  • Microscopy, Fluorescence
  • Molecular Sequence Data
  • Monomeric GTP-Binding Proteins / genetics
  • Monomeric GTP-Binding Proteins / metabolism*
  • Mutation
  • Myocytes, Cardiac / drug effects
  • Myocytes, Cardiac / metabolism*
  • Phosphorylation
  • Protein Transport
  • Recombinant Fusion Proteins / metabolism
  • Time Factors
  • Transfection

Substances

  • Calcium Channel Agonists
  • Calcium Channels, L-Type
  • L-type calcium channel alpha(1C)
  • Recombinant Fusion Proteins
  • 3-Pyridinecarboxylic acid, 1,4-dihydro-2,6-dimethyl-5-nitro-4-(2-(trifluoromethyl)phenyl)-, Methyl ester
  • Guanosine Triphosphate
  • Cyclic AMP-Dependent Protein Kinases
  • Monomeric GTP-Binding Proteins