Price range: $268.00 through $328.00
Background:
calcium voltage-gated channel subunit alpha1 S(CACNA1S) Homo sapiens This gene encodes one of the five subunits of the slowly inactivating L-type voltage-dependent calcium channel in skeletal muscle cells. Mutations in this gene have been associated with hypokalemic periodic paralysis, thyrotoxic periodic paralysis and malignant hyperthermia susceptibility. [provided by RefSeq, Jul 2008],
| Size | 50μL/100μL |
| Target | CACNA1S |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, ELISA |
| MW(Calculated) | |
| MW(Observed) | 206kD |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-2000; ELISA 1:5000-20000 |
| Formulation | Liquid in PBS containing 50% glycerol,0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| Immunogen | Synthesized peptide derived from human protein . at AA range: 330-410 |
| Specificity | CAC1S Polyclonal Antibody detects endogenous levels of protein. |
| Gene Name | CACNA1S CACH1 CACN1 CACNL1A3 |
| Protein Name | Voltage-dependent L-type calcium channel subunit alpha-1S (Calcium channel, L type, alpha-1 polypeptide, isoform 3, skeletal muscle) (Voltage-gated calcium channel subunit alpha Cav1.1) |
| Other Name | |
| Species | Human |
| Gene ID | 779 |
| Uniprot | Q13698, |
| Species.1 | Mouse |
| Gene ID.1 | |
| Uniprot.1 | Q02789, |
| Species.2 | Rat |
| Gene ID.2 | |
| Uniprot.2 | Q02485 |
| Cellular Localization | Cell membrane, sarcolemma, T-tubule; Multi-pass membrane protein . |
| Tissue Expression | Skeletal muscle specific. |
| Signaling_pathway | Organismal Systems >> Endocrine system >> Insulin secretion;Organismal Systems >> Endocrine system >> GnRH signaling pathway;Organismal Systems >> Endocrine system >> Oxytocin signaling pathway;Organismal Systems >> Endocrine system >> Growth hormone synthesis, secretion and action;Organismal Systems >> Endocrine system >> Aldosterone synthesis and secretion;Organismal Systems >> Circulatory system >> Cardiac muscle contraction;Organismal Systems >> Circulatory system >> Adrenergic signaling in cardiomyocytes;Organismal Systems >> Circulatory system >> Vascular smooth muscle contraction;Organismal Systems >> Nervous system >> Cholinergic synapse;Human Diseases >> Neurodegenerative disease >> Alzheimer disease;Human Diseases >> Neurodegenerative disease >> Prion disease;Human Diseases >> Neurodegenerative disease >> Pathways of neurodegeneration - multiple diseases;Environmental Information Processing >> Signal transduction >> MAPK signaling pathway;Environmental Information Processing >> Signal transduction >> Calcium signaling pathway;Environmental Information Processing >> Signal transduction >> cAMP signaling pathway;Environmental Information Processing >> Signal transduction >> cGMP-PKG signaling pathway |
| Research Areas | >>MAPK signaling pathway; / >>Calcium signaling pathway; / >>cGMP-PKG signaling pathway; / >>cAMP signaling pathway; / >>Cardiac muscle contraction; / >>Adrenergic signaling in cardiomyocytes; / >>Vascular smooth muscle contraction; / >>Retrograde endocannabinoid signaling; / >>Cholinergic synapse; / >>Serotonergic synapse; / >>GABAergic synapse; / >>Insulin secretion; / >>GnRH signaling pathway; / >>Oxytocin signaling pathway; / >>Renin secretion; / >>Aldosterone synthesis and secretion; / >>Cortisol synthesis and secretion; / >>GnRH secretion; / >>Cushing syndrome; / >>Growth hormone synthesis, secretion and action; / >>Alzheimer disease; / >>Prion disease; / >>Pathways of neurodegeneration - multiple diseases; / >>Chemical carcinogenesis - receptor activation; / >>Hypertrophic cardiomyopathy; / >>Arrhythmogenic right ventricular cardiomyopathy; / >>Dilated cardiomyopathy |
| Function | Disease:Defects in CACNA1S are a cause of periodic paralysis hypokalemic (HOKPP) [MIM:170400]; also designated HYPOPP. HOKPP is an autosomal dominant disorder manifested by episodic flaccid generalized muscle weakness associated with falls of serum potassium levels.,Disease:Defects in CACNA1S are the cause of malignant hyperthermia susceptibility 5 (MHS5) [MIM:601887]; an autosomal dominant disorder that is potentially lethal in susceptible individuals on exposure to commonly used inhalational anesthetics and depolarizing muscle relaxants.,Domain:Each of the four internal repeats contains five hydrophobic transmembrane segments (S1, S2, S3, S5, S6) and one positively charged transmembrane segment (S4). S4 segments probably represent the voltage-sensor and are characterized by a series of positively charged amino acids at every third position.,Domain:The loop between repeats II and III interacts with the ryanodine receptor, and is therefore important for calcium release from the endoplasmic reticulum necessary for muscle contraction.,Function:Voltage-sensitive calcium channels (VSCC) mediate the entry of calcium ions into excitable cells and are also involved in a variety of calcium-dependent processes, including muscle contraction, hormone or neurotransmitter release, gene expression, cell motility, cell division and cell death. The isoform alpha-1S gives rise to L-type calcium currents. Long-lasting (L-type) calcium channels belong to the 'high-voltage activated' (HVA) group. They are blocked by dihydropyridines (DHP), phenylalkylamines, benzothiazepines, and by omega-agatoxin-IIIA (omega-Aga-IIIA). They are however insensitive to omega-conotoxin-GVIA (omega-CTx-GVIA) and omega-agatoxin-IVA (omega-Aga-IVA). Calcium channels containing the alpha-1S subunit play an important role in excitation-contraction coupling in skeletal muscle.,PTM:Phosphorylation by PKA activates the calcium channel.,similarity:Belongs to the calcium channel alpha-1 subunit (TC 1.A.1.11) family.,subunit:Multisubunit complex consisting of alpha-1, alpha-2, beta and delta subunits in a 1:1:1:1 ratio. The channel activity is directed by the pore-forming and voltage-sensitive alpha-1 subunit. In many cases, this subunit is sufficient to generate voltage-sensitive calcium channel activity. The auxiliary subunits beta and alpha-2/delta linked by a disulfide bridge regulate the channel activity. An additional gamma subunit is present only in skeletal muscle L-type channel. Interacts with DYSF and JSRP1.,tissue specificity:Skeletal muscle specific., |
