RBM20 Rabbit pAb
- 产品详情
- 实验流程
Application
| WB, IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | Q5T481 |
| Other Accession | Q5T481 |
| Reactivity | Human, Mouse |
| Predicted | Rat, Dog, Horse, Sheep |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 134258 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human RBM20 |
| Epitope Specificity | 361-460/1227 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SUBCELLULAR LOCATION | Nucleus (By similarity). |
| SIMILARITY | Contains 1 RRM (RNA recognition motif) domain. |
| DISEASE | Defects in RBM20 are the cause of cardiomyopathy dilated type 1DD (CMD1DD) [MIM:613172]. A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | This gene encodes a protein that likely binds RNA. Mutations in this gene have been associated with familial dilated cardiomyopathy. [provided by RefSeq, Mar 2010] |
| Gene ID | 282996 |
|---|---|
| Other Names | 1110018J23Rik; 2010003H22Rik; RBM20_HUMAN; RBM20; RNA-binding motif protein 20; RBM20_MOUSE; RNA binding motif protein 20 |
| Target/Specificity | Expressed in the heart. |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,Flow-Cyt=1ug/test |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | RBM20 {ECO:0000303|PubMed:22466703, ECO:0000312|HGNC:HGNC:27424} |
|---|---|
| Function | RNA-binding protein that acts as a regulator of mRNA splicing of a subset of genes encoding key structural proteins involved in cardiac development, such as TTN (Titin), CACNA1C, CAMK2D or PDLIM5/ENH (PubMed:22466703, PubMed:24960161, PubMed:26604136, PubMed:27496873, PubMed:27531932, PubMed:29895960, PubMed:30948719, PubMed:32840935, PubMed:34732726, PubMed:35427468). Acts as a repressor of mRNA splicing: specifically binds the 5'UCUU-3' motif that is predominantly found within intronic sequences of pre-mRNAs, leading to the exclusion of specific exons in target transcripts (PubMed:24960161, PubMed:30948719, PubMed:34732726). RBM20-mediated exon skipping is hormone-dependent and is essential for TTN isoform transition in both cardiac and skeletal muscles (PubMed:27531932, PubMed:30948719). RBM20- mediated exon skipping of TTN provides substrates for the formation of circular RNA (circRNAs) from the TTN transcripts (PubMed:27531932, PubMed:34732726). Together with RBM24, promotes the expression of short isoforms of PDLIM5/ENH in cardiomyocytes (By similarity). |
| Cellular Location | Nucleus {ECO:0000255|PROSITE-ProRule:PRU00130, ECO:0000269|PubMed:23886709, ECO:0000269|PubMed:30262925, ECO:0000269|PubMed:32840935, ECO:0000269|PubMed:33188278, ECO:0000269|PubMed:34732726, ECO:0000269|PubMed:35427468}. Cytoplasm, Cytoplasmic ribonucleoprotein granule. Note=The active form that regulates alternative splicing localizes to the nucleus (PubMed:33188278, PubMed:34732726, PubMed:35427468). Also localizes to cytoplasmic ribonucleoprotein granules; localization to cytoplasmic ribonucleoprotein granules plays an important regulatory role (PubMed:33188278, PubMed:34732726, PubMed:35427468). Subcellular localization is regulated by phosphorylation of different parts of the protein: while phosphorylation of the RS (arginine/serine-rich) region promotes nuclear localization, phosphorylation of the C-terminal disordered region promotes localization to cytoplasmic ribonucleoprotein granules (PubMed:35427468) |
| Tissue Location | Mainly expressed in the heart (PubMed:19712804, PubMed:23886709). Also expressed in skeletal muscle tissues, ovary, small intestine and colon (PubMed:23886709) |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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