Rabbit anti-Human CSRP3 Polyclonal Antibody | anti-CSRP3 antibody
CSRP3 Antibody (Center)
domain proteins, which may be involved in regulatory processes
important for development and cellular differentiation. The
LIM/double zinc-finger motif found in this protein is found in a
group of proteins with critical functions in gene regulation, cell
growth, and somatic differentiation. Mutations in this gene are
thought to cause heritable forms of hypertrophic cardiomyopathy
(HCM) and dilated cardiomyopathy (DCM) in humans. Alternatively
spliced transcript variants with different 5' UTR, but encoding the
same protein, have been found for this gene.
Zimmerman, R.S., et al. Genet. Med. 12(5):268-278(2010)
Rampersaud, E., et al. Ann. Hum. Genet. 74(2):110-116(2010)
Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)
Moller, D.V., et al. Eur. J. Heart Fail. 11(11):1031-1035(2009)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a member of the CSRP family of LIM domain proteins, which may be involved in regulatory processes important for development and cellular differentiation. The LIM/double zinc-finger motif found in this protein is found in a group of proteins with critical functions in gene regulation, cell growth, and somatic differentiation. Mutations in this gene are thought to cause heritable forms of hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) in humans. Alternatively spliced transcript variants with different 5' UTR, but encoding the same protein, have been found for this gene. [provided by RefSeq, Jul 2008]
Uniprot Description
CSRP3: Positive regulator of myogenesis. Plays a crucial and specific role in the organization of cytosolic structures in cardiomyocytes. Could play a role in mechanical stretch sensing. May be a scaffold protein that promotes the assembly of interacting proteins at Z-line structures. It is essential for calcineurin anchorage to the Z line. Required for stress-induced calcineurin-NFAT activation. Defects in CSRP3 are the cause of cardiomyopathy dilated type 1M (CMD1M). 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 CSRP3 are the cause of familial hypertrophic cardiomyopathy type 12 (CMH12). Familial hypertrophic cardiomyopathy is a hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death.
Protein type: Transcription regulation
Chromosomal Location of Human Ortholog: 11p15.1
Cellular Component: cytoskeleton; nucleus; Z disc
Molecular Function: actinin binding; protein binding; telethonin binding; zinc ion binding; structural constituent of muscle
Biological Process: cellular calcium ion homeostasis; cardiac muscle development; skeletal muscle development; protein localization in organelle; positive regulation of transcription from RNA polymerase II promoter; cardiac myofibril assembly; regulation of the force of heart contraction; cardiac muscle contraction
Disease: Cardiomyopathy, Familial Hypertrophic, 12; Cardiomyopathy, Dilated, 1m
Research Articles on CSRP3
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Product Notes
The CSRP3 csrp3 (Catalog #AAA9210866) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 91-119. The CSRP3 Antibody (Center) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's CSRP3 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 CSRP3 csrp3 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 "CSRP3, 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
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