The ABCC9 Antibody (PAC020628) is a high-quality polyclonal antibody designed for research involving ABCC9, a cell surface protein that plays a crucial role in regulating cellular processes. This antibody, raised in rabbits, has been validated for use in Western blot applications and is highly reactive with human samples. ABCC9, also known as sulfonylurea receptor 2, is involved in ion transport and regulation of cell membrane potential. It is particularly important in the context of cardiovascular function, as mutations in ABCC9 have been linked to cardiovascular diseases such as dilated cardiomyopathy and hypertension.
This antibody binds specifically to ABCC9, enabling researchers to detect and analyze the protein in various cell types. It is an essential tool for studies in cardiovascular biology, pharmacology, and drug development, providing valuable insights into the mechanisms underlying cardiovascular diseases. Understanding the function of ABCC9 is key to developing targeted therapies for these conditions, making this antibody an invaluable resource for researchers in the field.
Antibody Name:
ABCC9 Antibody (PACO20628)
Antibody SKU:
PACO20628
Size:
50ul
Host Species:
Rabbit
Tested Applications:
ELISA, IHC
Recommended Dilutions:
ELISA:1:2000-1:5000, IHC:1:25-1:100
Species Reactivity:
Human, Mouse, Rat
Immunogen:
Synthetic peptide of human ABCC9
Form:
Liquid
Storage Buffer:
-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
Purification Method:
Antigen affinity purification
Clonality:
Polyclonal
Isotype:
IgG
Conjugate:
Non-conjugated
The image on the left is immunohistochemistry of paraffin-embedded Human esophagus cancer tissue using PACO20628(ABCC9 Antibody) at dilution 1/50, on the right is treated with synthetic peptide. (Original magnification: x200).
The image on the left is immunohistochemistry of paraffin-embedded Human liver cancer tissue using PACO20628(ABCC9 Antibody) at dilution 1/50, on the right is treated with synthetic peptide. (Original magnification: x200).
Background:
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.
Synonyms:
ATP-binding cassette, sub-family C (CFTR/MRP), member 9
UniProt Protein Function:
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, integral; Membrane protein, multi-pass; Transporter; Transporter, ABC family; Channel, potassiumChromosomal Location of Human Ortholog: 12p12.1Cellular Component: ATP-sensitive potassium channel complex; plasma membrane; voltage-gated potassium channel complexMolecular Function: anion transmembrane-transporting ATPase activity; potassium channel regulator activity; sulfonylurea receptor activity; transporter activityBiological Process: defense response to virus; potassium ion import; potassium ion transport; transmembrane transportDisease: Atrial Fibrillation, Familial, 12; Cantu Syndrome; Cardiomyopathy, Dilated, 1o
UniProt Protein Details:
NCBI Summary:
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]
