CRAC channelopathies

Ion Channels, Receptors and Transporters

Abstract

Store-operated Ca2+ entry (SOCE) is an important Ca2+ influx pathway in many non-excitable and some excitable cells. It is regulated by the filling state of intracellular Ca2+ stores, notably the endoplasmic reticulum (ER). Reduction in [Ca2+]ER results in activation of plasma membrane Ca2+ channels that mediate sustained Ca2+ influx which is required for many cell functions as well as refilling of Ca2+ stores. The Ca2+ release activated Ca2+ (CRAC) channel is the best characterized SOC channel with well-defined electrophysiological properties. In recent years, the molecular components of the CRAC channel, long mysterious, have been defined. ORAI1 (or CRACM1) acts as the pore-forming subunit of the CRAC channel in the plasma membrane. Stromal interaction molecule (STIM) 1 is localized in the ER, senses [Ca2+]ER, and activates the CRAC channel upon store depletion by binding to ORAI1. Both proteins are widely expressed in many tissues in both human and mouse consistent with the widespread prevalence of SOCE and CRAC channel currents in many cells types. CRAC channelopathies in human patients with mutations in STIM1 and ORAI1 are characterized by abolished CRAC channel currents, lack of SOCE and—clinically—immunodeficiency, congenital myopathy, and anhydrotic ectodermal dysplasia. This article reviews the role of ORAI and STIM proteins for SOCE and CRAC channel function in a variety of cell types and tissues and compares the phenotypes of ORAI1 and STIM1-deficient human patients and mice with targeted deletion of Orai and Stim genes.

Keywords

ORAI1 STIM1 CRAC SOCE Store-operated calcium entry Ca2+ T cells B cells Mast cells Lymphocytes Immunodeficiency SCID Signal transduction Myopathy Muscle Ectodermal dysplasia Amelogenesis Platelets Gene-targeting Mice 

Abbreviations

CRAC

Calcium release activated calcium (channel)

EDA

Anhydrotic ectodermal dysplasia

SCID

Severe combined immunodeficiency

SERCA

Sarcoplasmic/endoplasmic reticulum Ca2+ ATPase

SOCE

Store-operated calcium entry

STIM1

Stromal interaction molecule 1

VSMC

Vascular smooth muscle cells

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© Springer-Verlag 2010

Authors and Affiliations

  1. 1.Department of PathologyNew York University, Langone Medical Center, SRB314New YorkUSA
  2. 2.Department of PathologyNew York University, Langone Medical CenterNew YorkUSA

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