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Mouse Anti-ABCC6 Recombinant Antibody (V2-179037) (CBMAB-A0273-YC)

Provided herein is a Mouse monoclonal antibody against Human ATP Binding Cassette Subfamily C Member 6. The antibody can be used for immunoassay techniques, such as ELISA, WB.
See all ABCC6 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
V2-179037
Antibody Isotype
IgG2b, κ
Application
ELISA, WB

Basic Information

Immunogen
ABCC6 (NP_001162, 831 a.a. ~ 930 a.a) partial recombinant protein with GST tag.
Specificity
Human
Antibody Isotype
IgG2b, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
PBS, pH 7.2
Buffer
PBS, pH7.4
Preservative
None
Concentration
Batch dependent
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
ATP Binding Cassette Subfamily C Member 6
Introduction
ABCC6 is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN
Entrez Gene ID
UniProt ID
Alternative Names
ATP Binding Cassette Subfamily C Member 6; ATP-Binding Cassette, Sub-Family C (CFTR/MRP), Member 6; Anthracycline Resistance-Associated Protein; Multi-Specific Organic Anion Transporter E; MOAT-E; MRP6; ARA; ATP-Binding Cassette Sub-Family C Member 6; Mul
Function
Isoform 1: ATP-dependent transporter of the ATP-binding cassette (ABC) family that actively extrudes physiological compounds, and xenobiotics from cells. Mediates ATP-dependent transport of glutathione conjugates such as leukotriene-c4 (LTC4) and N-ethylmaleimide S-glutathione (NEM-GS) (in vitro), and an anionic cyclopentapeptide endothelin antagonist, BQ-123. Does not appear to actively transport drugs outside the cell. Confers low levels of cellular resistance to etoposide, teniposide, anthracyclines and cisplatin.Mediates the release of nucleoside triphosphates, predominantly ATP, into the circulation, where it is rapidly converted into AMP and the mineralization inhibitor inorganic pyrophosphate (PPi) by the ecto-enzyme ectonucleotide pyrophosphatase phosphodiesterase 1 (ENPP1), therefore playing a role in PPi homeostasis.
Isoform 2: Inhibits TNF-alpha-mediated apoptosis through blocking one or more caspases.
Biological Process
ATP transport
Cellular phosphate ion homeostasis
Drug transmembrane transport
Leukotriene transport
Response to drug
Transmembrane transport
Visual perception
Cellular Location
Isoform 1: Basolateral cell membrane
Isoform 2: Endoplasmic reticulum membrane
Involvement in disease
A multisystem disorder characterized by accumulation of mineralized and fragmented elastic fibers in the skin, vascular walls, and Burch membrane in the eye. Clinically, patients exhibit characteristic lesions of the posterior segment of the eye including peau d'orange, angioid streaks, and choroidal neovascularizations, of the skin including soft, ivory colored papules in a reticular pattern that predominantly affect the neck and large flexor surfaces, and of the cardiovascular system with peripheral and coronary arterial occlusive disease as well as gastrointestinal bleedings.
A severe autosomal recessive disorder characterized by calcification of the internal elastic lamina of muscular arteries and stenosis due to myointimal proliferation. The disorder is often fatal within the first 6 months of life because of myocardial ischemia resulting in refractory heart failure.
Topology
Extracellular: 1-31 aa
Helical: 32-52 aa
Cytoplasmic: 53-72 aa
Helical: 73-93 aa
Extracellular: 94-98 aa
Helical: 99-119 aa
Cytoplasmic: 120-131 aa
Helical: 132-149 aa
Extracellular: 150-167 aa
Helical: 168-188 aa
Cytoplasmic: 189-302 aa
Helical: 303-323 aa
Extracellular: 324-349 aa
Helical: 350-370 aa
Cytoplasmic: 371-426 aa
Helical: 427-447 aa
Extracellular: 448-450 aa
Helical: 451-471 aa
Cytoplasmic: 472-533 aa
Helical: 534-554 aa
Extracellular: 555-575 aa
Helical: 576-596 aa
Cytoplasmic: 597-939 aa
Helical: 940-960 aa
Extracellular: 961-997 aa
Helical: 998-1018 aa
Cytoplasmic: 1019-1061 aa
Helical: 1062-1082 aa
Extracellular: 1083 aa
Helical: 1084-1104 aa
Cytoplasmic: 1105-1175 aa
Helical: 1176-1196 aa
Extracellular: 1197-1198 aa
Helical: 1199-1219 aa
Cytoplasmic: 1220-1503 aa
PTM
Glycosylated.

Szeri, F., Corradi, V., Niaziorimi, F., Donnelly, S., Conseil, G., Cole, S. P., ... & van de Wetering, K. (2021). Mutagenic Analysis of the Putative ABCC6 Substrate-Binding Cavity Using a New Homology Model. International Journal of Molecular Sciences, 22(13), 6910.

Bisaccia, F., Koshal, P., Abruzzese, V., Castiglione Morelli, M. A., & Ostuni, A. (2021). Structural and Functional Characterization of the ABCC6 Transporter in Hepatic Cells: Role on PXE, Cancer Therapy and Drug Resistance. International Journal of Molecular Sciences, 22(6), 2858.

Verschuere, S., Van Gils, M., Nollet, L., & Vanakker, O. M. (2020). From membrane to mineralization: the curious case of the ABCC6 transporter. FEBS letters, 594(23), 4109-4133.

Tiemann, J., Wagner, T., Lindenkamp, C., Plümers, R., Faust, I., Knabbe, C., & Hendig, D. (2020). Linking ABCC6 Deficiency in Primary Human Dermal Fibroblasts of PXE Patients to p21-Mediated Premature Cellular Senescence and the Development of a Proinflammatory Secretory Phenotype. International Journal of Molecular Sciences, 21(24), 9665.

De Vilder, E. Y., Cardoen, S., Hosen, M. J., Le Saux, O., De Zaeytijd, J., Leroy, B. P., ... & Vanakker, O. M. (2018). Pathogenic variants in the ABCC6 gene are associated with an increased risk for ischemic stroke. Brain Pathology, 28(6), 822-831.

Martinelli, F., Cuviello, F., Pace, M. C., Armentano, M. F., Miglionico, R., Ostuni, A., & Bisaccia, F. (2018). Extracellular ATP regulates CD73 and ABCC6 expression in HepG2 cells. Frontiers in molecular biosciences, 5, 75.

Akhter, S., Georgas, T., & Giannoukos, G. (2018). Investigating Mutations in Member 6 (ABCC6) gene, of Sub-Family C (CFTR/MRP) of the ATP-binding Cassette Causing Pseudoxanthoma elasficum. J Biol Med Res, 2(2), 17.

Colleoni, L., Galbardi, B., Barzago, C., Bonanno, S., Franzi, S., Frangiamore, R., ... & Kapetis, D. (2017). A novel ABCC6 haplotype is associated with azathioprine drug response in myasthenia gravis. Pharmacogenetics and genomics, 27(2), 51-56.

Miglionico, R., Ostuni, A., Armentano, M. F., Milella, L., Crescenzi, E., Carmosino, M., & Bisaccia, F. (2017). ABCC6 knockdown in HepG2 cells induces a senescent-like cell phenotype. Cellular & molecular biology letters, 22(1), 1-10.

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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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