Price range: $268.00 through $328.00
Background:
The protein encoded by this gene belongs to the family of P-type cation transport ATPases. This magnesium-dependent enzyme catalyzes the hydrolysis of ATP coupled with the transport of calcium ions. Defects in this gene cause Hailey-Hailey disease, an autosomal dominant disorder. Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Aug 2011],
| Size | 50μL/100μL |
| Target | PMR1 |
| Reactivity | Human, Monkey |
| Applications | WB, IHC, IF, ELISA |
| MW(Calculated) | 101kD |
| MW(Observed) | |
| Host Species | Mouse |
| Isotype | |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-1:2000; IHC 1:200-1:1000; ELISA 1:10000; IF 1:50-200 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Purification | Affinity purification |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | |
| Clonality | Monoclonal |
| Clone Number | 18B6 |
| Immunogen | Purified recombinant fragment of PMR1 expressed in E. Coli. |
| Specificity | PMR1 Monoclonal Antibody detects endogenous levels of PMR1 protein. |
| Gene Name | ATP2C1 |
| Protein Name | Calcium-transporting ATPase type 2C member 1 |
| Other Name | ATP2C1; / KIAA1347; / PMR1L; / HUSSY-28; / Calcium-transporting ATPase type 2C member 1; / ATPase 2C1; / ATP-dependent Ca; / 2+; / pump PMR1 |
| Species | Human |
| Gene ID | 27032 |
| Uniprot | P98194, |
| Species.1 | Mouse |
| Gene ID.1 | |
| Uniprot.1 | Q80XR2 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Golgi apparatus, trans-Golgi network membrane; Multi-pass membrane protein . Golgi apparatus, Golgi stack membrane; Multi-pass membrane protein . During neuron differentiation, shifts from juxtanuclear Golgi position to multiple Golgi structures distributed over the neural soma with a predominance in the apical dendritic trunk. . |
| Tissue Expression | Found in most tissues except colon, thymus, spleen and leukocytes (PubMed:15831496). Expressed in keratinocytes (at protein level) (PubMed:15831496, PubMed:14632183). |
| Signaling_pathway | |
| Research Areas | |
| Function | Alternative products:Isoform 1 and isoform 2 are expressed in the same tissues,Catalytic activity:ATP + H(2)O + Ca(2+)(Cis) = ADP + phosphate + Ca(2+)(Trans).,Disease:Defects in ATP2C1 are the cause of Hailey-Hailey disease (HHD) [MIM:169600]; also known as benign familial pemphigus. HHD is an autosomal dominant disorder characterized by persistent blisters and suprabasal cell separation (acantholysis) of the epidermis, due to impaired keratinocyte adhesion. Patients lacking all isoforms except isoform 2 have HHD.,Function:This magnesium-dependent enzyme catalyzes the hydrolysis of ATP coupled with the transport of the calcium.,similarity:Belongs to the cation transport ATPase (P-type) family.,similarity:Belongs to the cation transport ATPase (P-type) family. Type IIA subfamily.,tissue specificity:Found in most tissues except colon, thymus, spleen and leukocytes. Most abundant in keratinocytes and kidney., |
