anti- ABCC6 antibody
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Description
The ATP-binding cassette transporter 6 (ABCC6) gene, also named as MRP6, is a cellular transmembrane protein transporter and belongs to ATP-binding cassette (ABC) family. ABCC6 is an ATP-dependent transporter contains two ATP-binding domains. It is mainly found in the liver, the proximal tubules of the kidneys and the intestines. ABCC6 is involved in a pathway of extracellular nucleotide metabolism and the regulation of tissue calcification. Mutations of ABCC6 leads to pseudoxanthoma elasticum (PXE) which is a rare autosomal recessive disorder. Mutations of ABCC6 can also lead generalized arterial calcification of infancy-2 (GACI2).
Documents del producto
Product specifications
| Category | Primary Antibodies |
| Immunogen Target | ATP-binding cassette, sub-family C (CFTR/MRP), member 6 |
| Host | Rabbit |
| Reactivity | human |
| Recommended Dilution | IHC: 1:20-1:200 |
| Clonality | polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Observed MW | 165 kDa |
| Purity | ≥95% as determined by SDS-PAGE |
| Purification | Immunogen affinity purified |
| Size 1 | 100µg |
| Form | liquid |
| Tested Applications | ELISA, IHC |
| Storage | PBS with 0.02% sodium azide and 50% glycerol pH 7.3,-20℃ for 12 months(Avoid repeated freeze / thaw cycles.) |
| UniProt ID | O95255 |
| Alias | ABC34,ARA,EST349056,GACI2,MLP1,MOAT-E,Multidrug resistance-associated protein 6 |
| Background | Antibody anti-ABCC6 |
| Status | RUO |
| Note | This product is for research use only. |
Descripción
ATP Binding Cassette Subfamily C Member 6 (ABCC6) is primarily recognized for its involvement in regulating tissue calcification processes. Mutations in the ABCC6 gene are linked to pseudoxanthoma elasticum (PXE), a rare genetic disorder characterized by progressive mineralization of elastic fibers in the skin, eyes, and cardiovascular system. ABCC6 functions as an efflux transporter for ATP metabolites, such as inorganic pyrophosphate (PPi), a critical inhibitor of pathological calcification. Expressed predominantly in the liver and kidneys, ABCC6 indirectly regulates extracellular matrix homeostasis and mineralization processes in peripheral tissues. The transporter’s activity impacts not only PXE pathology but also vascular calcification seen in chronic kidney disease and atherosclerosis. ABCC6 remains a focal point of research for developing targeted therapies to modulate its function and mitigate disease progression, emphasizing its importance in both genetic and acquired calcification disorders.
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