CSRP3 Antibody

說明書

中文名稱：

CSRP3兔多克隆抗體
貨號：

CSB-PA006087ESR1HU
規格：

￥440
圖片：
Immunohistochemistry of paraffin-embedded human heart tissue using CSB-PA006087ESR1HU at dilution of 1:100

Immunohistochemistry of paraffin-embedded human skeletal muscle tissue using CSB-PA006087ESR1HU at dilution of 1:100
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) CSRP3 Polyclonal antibody
Uniprot No.：

P50461
基因名：

CSRP3
別名：

cardiac antibody; Cardiac LIM protein antibody; CLP antibody; CMD1M antibody; CMH12 antibody; CRP3 antibody; Csrp3 antibody; CSRP3_HUMAN antibody; Cysteine and glycine-rich protein 3 antibody; Cysteine rich protein 3 antibody; Cysteine-rich protein 3 antibody; LIM domain only 4 antibody; LIM domain protein antibody; LMO4 antibody; MLP antibody; Muscle LIM protein antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Cysteine and glycine-rich protein 3 protein (1-194AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
產品提供形式：

Liquid
應用范圍：

ELISA, IHC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:20-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

Positive regulator of myogenesis. Acts as cofactor for myogenic bHLH transcription factors such as MYOD1, and probably MYOG and MYF6. Enhances the DNA-binding activity of the MYOD1:TCF3 isoform E47 complex and may promote formation of a functional MYOD1:TCF3 isoform E47:MEF2A complex involved in 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. The role in regulation of cytoskeleton dynamics by association with CFL2 is reported conflictingly: Shown to enhance CFL2-mediated F-actin depolymerization dependent on the CSRP3:CFL2 molecular ratio, and also shown to reduce the ability of CLF1 and CFL2 to enhance actin depolymerization. Proposed to contribute to the maintenance of muscle cell integerity through an actin-based mechanism. Can directly bind to actin filaments, cross-link actin filaments into bundles without polarity selectivity and protect them from dilution- and cofilin-mediated depolymerization; the function seems to involve its self-association. In vitro can inhibit PKC/PRKCA activity. Proposed to be involved in cardiac stress signaling by down-regulating excessive PKC/PRKCA signaling.; May play a role in early sarcomere organization. Overexpression in myotubes negatively regulates myotube differentiation. By association with isoform 1 and thus changing the CSRP3 isoform 1:CFL2 stoichiometry is proposed to down-regulate CFL2-mediated F-actin depolymerization.
基因功能參考文獻：
1. Previous results along with the newly identified homozygous CSRP3 truncating variants in two unrelated hypertrophic cardiomyopathy (HCM) patients suggest that the association of CSRP3 as a validated HCM-causing gene require additional studies and those CSRP3 variants could result in HCM with an autosomal recessive inheritance rather than with an autosomal dominant transmission as usually reported on HCM. PMID: 30012424
2. MLP contributes to the maintenance of cardiomyocyte cytoarchitecture by a mechanism involving its self-association and actin filament cross-linking. PMID: 24934443
3. study reports the discovery of an alternative splice variant of muscle lim protein encoded by the CSRP3 gene, designated as MLP-b, showing distinct expression in neuromuscular disease and direct roles in actin dynamics and muscle differentiation PMID: 24860983
4. KLF5 reverses hhLIM function from anti-proliferation to pro-proliferation through its interaction with hhLIM on the cyclin E promoter. PMID: 22584587
5. The CSRP3-W4R mutation causes cardiomyopathy and heart failure in patients and engineered knock-in animals. PMID: 20044516
6. CSRP3 is involved in cardiac mechanosensory processes, is localized to the sarcomeric Z-disc and human mutations cause cardiomyopathy(DCM)and heart failure. PMID: 12507422
7. Mutations in the CRP3/MLP gene can cause hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM). PMID: 12642359
8. CSRP3, MUSTN1, SIX1, and FBXO32 expression changes in response to lengthening and shortening contractions in human muscle PMID: 17519359
9. A myocardial actin-binding protein that increases actin cytoskeleton stability by promoting bundling of actin filaments. PMID: 18331358
10. These findings suggest that hhLIM is a typical LIM family member with powerful transcription activation. PMID: 18393774
11. Study used linkage analysis and identified a CSRP3 missense mutation in a large German family affected by hypertrophic cardiomyopathy. PMID: 18505755
12. CSRP3 mutation was found involved in hypertrophic cardiomyopathy. PMID: 19035361
13. The structure of both LIM domains of human MLP by nuclear magnetic resonance spectroscopy. PMID: 19230835
14. CRP3/MLP is primarily expressed in arterial smooth muscle cells and that stretch is the main stimulus for CRP3/MLP induction in veins exposed to arterial haemodynamic conditions. PMID: 19351738
15. Complete chemical shift assignment was achieved for the first LIM domain and for most of the second domain, the N-terminal and C-terminal linker and part of the intervening linker. PMID: 19636821
16. MLP binds directly to CFL2 in human cardiac and skeletal muscles. PMID: 19752190
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相關疾病：

Cardiomyopathy, dilated 1M (CMD1M); Cardiomyopathy, familial hypertrophic 12 (CMH12)
亞細胞定位：

Nucleus. Cytoplasm. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line. Cytoplasm, myofibril, sarcomere.; [Isoform 2]: Cytoplasm, myofibril, sarcomere, Z line.
組織特異性：

Cardiac and slow-twitch skeletal muscles. Isoform 2 is expressed in striated muscle. Isoform 2 is specifically expressed at higher levels in patients with neuromuscular diseases, such as limb-girdle muscular dystrophy 2A (LGMD2A), Duchenne muscular dystro
數據庫鏈接：

HGNC: 2472

OMIM: 600824

KEGG: hsa:8048

STRING: 9606.ENSP00000265968

UniGene: Hs.83577

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