Western Blot (WB)
(ABCC9 Antibody (Center) western blot analysis in mouse kidney tissue lysates (35ug/lane).This demonstrates the ABCC9 Antibody detected the ABCC9 protein (arrow).)
Rabbit anti-Human, mouse ABCC9 Polyclonal Antibody | anti-ABCC9 antibody
ABCC9 Antibody (Center)
Western Blot (WB)
(ABCC9 Antibody (Center) western blot analysis in mouse kidney tissue lysates (35ug/lane).This demonstrates the ABCC9 Antibody detected the ABCC9 protein (arrow).)
Western Blot (WB)
(ABCC9 Antibody (Center) western blot analysis in mouse kidney tissue lysates (35ug/lane).This demonstrates the ABCC9 Antibody detected the ABCC9 protein (arrow).)
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, GCN20, White). This
protein is a member of the MRP subfamily which is involved in
multi-drug resistance. This protein is thought to form
ATP-sensitive potassium channels in cardiac, skeletal, and vascular
and non-vascular smooth muscle. Protein structure suggests a role
as the drug-binding channel-modulating subunit of the
extrapancreatic ATP-sensitive potassium channels. No disease has
been associated with this gene thus far. Alternative splicing of
this gene results in several products, two of which result from
differential usage of two terminal exons and one of which results
from exon deletion.
Zimmerman, R.S., et al. Genet. Med. 12(5):268-278(2010)
Ellis, J.A., et al. Physiol. Genomics 40(3):184-188(2010)
Kim, S.J., et al. Biochem. Biophys. Res. Commun. 391(1):974-978(2010)
Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene 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, GCN20, White). This protein is a member of the MRP subfamily which is involved in multi-drug resistance. This protein is thought to form ATP-sensitive potassium channels in cardiac, skeletal, and vascular and non-vascular smooth muscle. Protein structure suggests a role as the drug-binding channel-modulating subunit of the extra-pancreatic ATP-sensitive potassium channels. Mutations in this gene are associated with cardiomyopathy dilated type 1O. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2011]
Uniprot Description
ABCC9: Subunit of ATP-sensitive potassium channels (KATP). Can form cardiac and smooth muscle-type KATP channels with KCNJ11. KCNJ11 forms the channel pore while ABCC9 is required for activation and regulation. Defects in ABCC9 are the cause of cardiomyopathy dilated type 1O (CMD1O); also known as dilated cardiomyopathy with ventricular tachycardia. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. Defects in ABCC9 are the cause of familial atrial fibrillation type 12 (ATFB12). ATFB12 is a familial form of atrial fibrillation, a common sustained cardiac rhythm disturbance. Atrial fibrillation is characterized by disorganized atrial electrical activity and ineffective atrial contraction promoting blood stasis in the atria and reduces ventricular filling. It can result in palpitations, syncope, thromboembolic stroke, and congestive heart failure. Defects in ABCC9 are the cause of hypertrichotic osteochondrodysplasia (HTOCD). A rare disorder characterized by congenital hypertrichosis, neonatal macrosomia, a distinct osteochondrodysplasia, and cardiomegaly. The hypertrichosis leads to thick scalp hair, which extends onto the forehead, and a general increase in body hair. In addition, macrocephaly and coarse facial features, including a broad nasal bridge, epicanthal folds, a wide mouth, and full lips, can be suggestive of a storage disorder. About half of affected individuals are macrosomic and edematous at birth, whereas in childhood they usually have a muscular appearance with little subcutaneous fat. Thickened calvarium, narrow thorax, wide ribs, flattened or ovoid vertebral bodies, coxa valga, osteopenia, enlarged medullary canals, and metaphyseal widening of long bones have been reported. Cardiac manifestations such as patent ductus arteriosus, ventricular hypertrophy, pulmonary hypertension, and pericardial effusions are present in approximately 80% of cases. Motor development is usually delayed due to hypotonia. Most patients have a mild speech delay, and a small percentage have learning difficulties or intellectual disability. Belongs to the ABC transporter superfamily. ABCC family. Conjugate transporter (TC 3.A.1.208) subfamily. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Membrane protein, multi-pass; Channel, potassium; Membrane protein, integral; Transporter; Transporter, ABC family
Chromosomal Location of Human Ortholog: 12p12.1
Cellular Component: voltage-gated potassium channel complex; sarcomere; ATP-sensitive potassium channel complex; plasma membrane; sarcolemma
Molecular Function: potassium channel regulator activity; potassium channel activity; ATPase activity, coupled to transmembrane movement of substances; sulfonylurea receptor activity; transporter activity; ATP binding
Biological Process: synaptic transmission; potassium ion import; metabolic process; signal transduction; transmembrane transport; defense response to virus; potassium ion transport
Disease: Cantu Syndrome; Cardiomyopathy, Dilated, 1o; Atrial Fibrillation, Familial, 12
Research Articles on ABCC9
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Product Notes
The ABCC9 abcc9 (Catalog #AAA9211663) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 640-669. The ABCC9 Antibody (Center) reacts with Human, mouse and may cross-react with other species as described in the data sheet. AAA Biotech's ABCC9 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB~~1:1000. Researchers should empirically determine the suitability of the ABCC9 abcc9 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "ABCC9, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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