Calsequestrin (CASQ) is a main calcium binding protein located within the lumen of sarcoplasmic reticulum .
Regulation of calcium binding proteins calreticulin and calsequestrin during differentiation in the myogenic cell line L6.
Beard et al., "Antibodies targeting the calcium binding skeletal muscle protein calsequestrin
are specific markers of ophthalmopathy and sensitive indicators of ocular myopathy in patients with Graves' disease," Clinical & Experimental Immunology, vol.
Gene Protein Mutation Donor cell types CASQ2 Calsequestrin
2 p.D307H Fibroblasts RyR2 Ryanodine receptor 2 p.P2328S Fibroblasts RyR2 Ryanodine receptor 2 p.F2483I Fibroblasts RyR2 Ryanodine receptor 2 p.S406L Fibroblasts Gene Reprogramming method References CASQ2 Lv (a), OSKM (b)  RyR2 RV (c), OSKM  RyR2 RV, OSKM  RyR2 RV, OSKM  LV (a): lentiviruses, RV (c): retroviruses, and OCT4 (O), SOX2 (S), KLF4 (K), and c-MYC (M) (b).
The researchers developed a mouse model for CPVT (by eliminating the calsequestrin
gene) and proposed using the model to study medications and interventions for the disorder.
Staining of the [Ca.sup.2+]-binding proteins, calsequestrin
, calmodulin, troponin C, and S-100, with the cationic carbocyanine dye "Stains-all".
Patients selected for the study had effort-induced polymorphic ventricular arrhythmias; this symptom was recently also found to be associated with mutations in the cardiac calsequestrin
gene (CASQ2; OMIM 114251) (37).
type-1 CASQ1-null: CASQ1 knockout mice CAT: Catalase EC coupling: Excitation-contraction coupling EDL: Extensor digitorum longus FDB: Flexor digitorum brevis GSH: Reduced glutathione GSSG: Oxidized glutathione [H.sub.2][O.sub.2]: Hydrogen peroxide IVCT: In vitro contracture test NO: Nitric oxide nNOS: Neuronal nitric oxide synthase NOX2: NADPH oxidase gp91phox membrane-bound subunit MH: Malignant hyperthermia [O.sub.2.sup.-]: Superoxide anion ROS: Reactive species of oxygen RNS: Reactive species of nitrogen RYR: Ryanodine receptor SOD1: Copper/zinc superoxide dismutase SOD2: Manganese superoxide dismutase SR: Sarcoplasmic reticulum.
These data are further supported, in part, with the fact that a [Zn.sup.2+]-binding protein calsequestrin
resides in the SR and can bind up to 200 mol [Zn.sup.2+] per mol, independently of binding up to 50 mol [Ca.sup.2+] per mol calsequestrin