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Mouse Anti-ABCC8 Recombinant Antibody (V2-179044) (CBMAB-A0280-YC)

Provided herein is a Mouse monoclonal antibody against Human ATP Binding Cassette Subfamily C Member 8. The antibody can be used for immunoassay techniques, such as ELISA, WB.
See all ABCC8 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
V2-179044
Antibody Isotype
IgG2a, κ
Application
ELISA, WB

Basic Information

Immunogen
Partial recombinant corresponding to aa611-711 from human ABCC8 (NP_000343) with GST tag.
Specificity
Human
Antibody Isotype
IgG2a, 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
Liquid in 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, sub-family C (CFTR/MRP), member 8
Introduction
ABCC8 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
6833
UniProt ID
Q09428
Alternative Names
ATP Binding Cassette Subfamily C Member 8; ATP-Binding Cassette, Sub-Family C (CFTR/MRP), Member 8; Sulfonylurea Receptor (Hyperinsulinemia); Sulfonylurea Receptor 1; HRINS; SUR1; SUR; ATP-Binding Cassette Transporter Sub-Family C Member 8; ATP-Binding Ca
Function
Subunit of the beta-cell ATP-sensitive potassium channel (KATP). Regulator of ATP-sensitive K+ channels and insulin release.
Biological Process
Inorganic cation transmembrane transport
Potassium ion transmembrane transport
Potassium ion transport
Regulation of insulin secretion
Transmembrane transport
Cellular Location
Cell membrane
Involvement in disease
Rare cause of hypoglycemia and is described as a condition in which symptomatic hypoglycemia is provoked by high protein feedings. Hypoglycemia is also elicited by administration of oral or intravenous infusions of a single amino acid, leucine.
Most common cause of persistent hypoglycemia in infancy. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur.
A form of permanent neonatal diabetes mellitus, a type of diabetes characterized by onset of persistent hyperglycemia within the first six months of life. Initial clinical manifestations include intrauterine growth retardation, hyperglycemia, glycosuria, osmotic polyuria, severe dehydration, and failure to thrive. Some PNDM3 patients may also have developmental delay, muscle weakness, and epilepsy. PNDM3 transmission pattern is consistent with autosomal dominant or autosomal recessive inheritance.
Neonatal diabetes is a form of diabetes mellitus defined by the onset of mild-to-severe hyperglycemia within the first months of life. Transient neonatal diabetes remits early, with a possible relapse during adolescence.
Topology
Extracellular: 1-34 aa
Helical: 35-55 aa
Cytoplasmic: 56-75 aa
Helical: 76-96 aa
Extracellular: 97-101 aa
Helical: 102-122 aa
Cytoplasmic: 123-134 aa
Helical: 135-154 aa
Extracellular: 155-167 aa
Helical: 168-194 aa
Cytoplasmic: 195-311 aa
Helical: 312-331 aa
Extracellular: 332-355 aa
Helical: 356-376 aa
Cytoplasmic: 377-434 aa
Helical: 435-455 aa
Extracellular: 456-458 aa
Helical: 459-479 aa
Cytoplasmic: 480-541 aa
Helical: 542-562 aa
Extracellular: 563-584 aa
Helical: 585-605 aa
Cytoplasmic: 606-1004 aa
Helical: 1005-1025 aa
Extracellular: 1026-1072 aa
Helical: 1073-1093 aa
Cytoplasmic: 1094-1137 aa
Helical: 1138-1158 aa
Extracellular: 1159 aa
Helical: 1160-1180 aa
Cytoplasmic: 1181-1251 aa
Helical: 1252-1272 aa
Extracellular: 1273-1276 aa
Helical: 1277-1297 aa
Cytoplasmic: 1298-1581 aa

Giannopoulou, E. Z., Ovcarov, O., De Franco, E., Kassberger, F., Nusser, S., Otto, M. C., ... & Wabitsch, M. (2021). Transient neonatal diabetes due to a disease causing novel variant in the ATP-binding cassette subfamily C member 8 (ABCC8) gene unmasks maturity-onset diabetes of the young (MODY) diabetes cases within a family. Journal of Pediatric Endocrinology and Metabolism, 34(2), 273-276.

Timmers, M., Dirinck, E., Lauwers, P., Wuyts, W., & De Block, C. (2021). ABCC8 variants in MODY12: Review of the literature and report of a case with severe complications. Diabetes/Metabolism Research and Reviews, e3459.

Mamoor, S. (2020). ATP-binding cassette transporter sub-family C member 8, ABCC8, is a differentially expressed gene in brain metastatic human breast cancer.

Lin, L., Quan, H., Chen, K., Chen, D., Lin, D., & Fang, T. (2020). ABCC8-Related Maturity-Onset Diabetes of the Young (MODY12): A Report of a Chinese Family. Frontiers in Endocrinology, 11, 645.

Lago-Docampo, M., Tenorio, J., Hernández-González, I., Pérez-Olivares, C., Escribano-Subías, P., Pousada, G., ... & Valverde, D. (2020). Characterization of rare ABCC8 variants identified in Spanish pulmonary arterial hypertension patients. Scientific reports, 10(1), 1-13.

Koufakis, T., Sertedaki, A., Tatsi, E. B., Trakatelli, C. M., Karras, S. N., Manthou, E., ... & Kotsa, K. (2019). First report of diabetes phenotype due to a loss-of-function ABCC8 mutation previously known to cause congenital hyperinsulinism. Case reports in genetics, 2019.

Shima, K. R., Usuda, R., Futatani, T., Akahori, H., Kaneko, S., Yorifuji, T., & Takamura, T. (2018). Heterogeneous nature of diabetes in a family with a gain-of-function mutation in the ATP-binding cassette subfamily C member 8 (ABCC8) gene. Endocrine journal, EJ18-0054.

Yamazaki, M., Sugie, H., Oguma, M., Yorifuji, T., Tajima, T., & Yamagata, T. (2017). Sulfonylurea treatment in an infant with transient neonatal diabetes mellitus caused by an adenosine triphosphate binding cassette subfamily C member 8 gene mutation. Clinical Pediatric Endocrinology, 26(3), 165-169.

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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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