Description
| 抗体名: | CSRP3 Antibody (PACO08640) |
| 抗体コード: | PACO08640 |
| サイズ: | 50ul |
| 宿主種: | Rabbit |
| 申し込み: | ELISA, WB, IHC |
| 推奨される希釈: | |
| 反応性: | Human, Mouse, Rat |
| 免疫原: | Human CSRP3 |
| 憲法: | Liquid |
| ストレージバッファ: | PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles. |
| 精製方法: | Antigen Affinity Purified |
| 抗体のクローン性: | Polyclonal |
| アイソタイプ: | IgG |
| Conjugate: | Non-conjugated |
| シノニム: | cysteine and glycine-rich protein 3 (cardiac LIM protein);CSRP3;CLP;CMD1M;CMH12;CRP3;LMO4;MGC14488;MGC61993;MLP ; |
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | Protein type:Transcription regulation Chromosomal Location of Human Ortholog: 11p15.1 Cellular Component: Z disc Molecular Function:actinin binding; protein binding; structural constituent of muscle; telethonin binding Biological Process: cardiac muscle contraction; cardiac muscle development; cardiac myofibril assembly; cellular calcium ion homeostasis; positive regulation of transcription from RNA polymerase II promoter; protein localization in organelle; regulation of the force of heart contraction; skeletal muscle development Disease: Cardiomyopathy, Dilated, 1m; Cardiomyopathy, Familial Hypertrophic, 12 |
| NCBI Summary: | 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 Code: | P50461 |
| NCBI GenInfo Identifier: | 1705933 |
| NCBI Gene ID: | 8048 |
| NCBI Accession: | P50461.1 |
| UniProt Secondary Accession: | P50461,Q9P131, |
| UniProt Related Accession: | P50461 |
| Molecular Weight: | 20,969 Da |
| NCBI Full Name: | Cysteine and glycine-rich protein 3 |
| NCBI Synonym Full Names: | cysteine and glycine rich protein 3 |
| NCBI Official Symbol: | CSRP3 |
| NCBI Official Synonym Symbols: | CLP; MLP; CRP3; LMO4; CMD1M; CMH12 |
| NCBI Protein Information: | cysteine and glycine-rich protein 3 |
| UniProt Protein Name: | Cysteine and glycine-rich protein 3 |
| UniProt Synonym Protein Names: | Cardiac LIM protein; Cysteine-rich protein 3; CRP3; LIM domain protein, cardiac; Muscle LIM protein |
| Protein Family: | Cysteine and glycine-rich protein |
| UniProt Gene Name: | CSRP3 |
| UniProt Entry Name: | CSRP3_HUMAN |
