Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) CSRP3 Polyclonal antibody

Uniprot No.

P50461

Target Names

CSRP3

Alternative Names

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

Raised in

Rabbit

Species Reactivity

Human, Mouse

Immunogen

Recombinant Human Cysteine and glycine-rich protein 3 protein (1-194AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Clonality

Polyclonal

Isotype

IgG

Purification Method

Antigen Affinity Purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Form

Liquid

Tested Applications

ELISA, WB, IHC

Recommended Dilution

Application	Recommended Dilution
WB	1:1000-1:5000
IHC	1:20-1:200

Protocols

ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

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

Lead Time

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.

Target Background

Function

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.

Gene References into Functions

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
MLP contributes to the maintenance of cardiomyocyte cytoarchitecture by a mechanism involving its self-association and actin filament cross-linking. PMID: 24934443
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
KLF5 reverses hhLIM function from anti-proliferation to pro-proliferation through its interaction with hhLIM on the cyclin E promoter. PMID: 22584587
The CSRP3-W4R mutation causes cardiomyopathy and heart failure in patients and engineered knock-in animals. PMID: 20044516
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
Mutations in the CRP3/MLP gene can cause hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM). PMID: 12642359
CSRP3, MUSTN1, SIX1, and FBXO32 expression changes in response to lengthening and shortening contractions in human muscle PMID: 17519359
A myocardial actin-binding protein that increases actin cytoskeleton stability by promoting bundling of actin filaments. PMID: 18331358
These findings suggest that hhLIM is a typical LIM family member with powerful transcription activation. PMID: 18393774
Study used linkage analysis and identified a CSRP3 missense mutation in a large German family affected by hypertrophic cardiomyopathy. PMID: 18505755
CSRP3 mutation was found involved in hypertrophic cardiomyopathy. PMID: 19035361
The structure of both LIM domains of human MLP by nuclear magnetic resonance spectroscopy. PMID: 19230835
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
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
MLP binds directly to CFL2 in human cardiac and skeletal muscles. PMID: 19752190

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Involvement in disease

Cardiomyopathy, dilated 1M (CMD1M); Cardiomyopathy, familial hypertrophic 12 (CMH12)

Subcellular Location

Nucleus. Cytoplasm. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line. Cytoplasm, myofibril, sarcomere.; [Isoform 2]: Cytoplasm, myofibril, sarcomere, Z line.

Tissue Specificity

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

Database Links

HGNC: 2472

OMIM: 600824

KEGG: hsa:8048

STRING: 9606.ENSP00000265968

UniGene: Hs.83577

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Code	CSB-PA006087ESR2HU
Size	US$166
	Order now
Image	Western blot All lanes: CSRP3 antibody at 1μg/ml + Mouse skeletal muscle tissue Secondary Goat polyclonal to rabbit IgG at 1/10000 dilution Predicted band size: 21 kDa Observed band size: 21, 25 kDa Immunohistochemistry of paraffin-embedded human heart tissue using CSB-PA006087ESR2HU at dilution of 1:100 Immunohistochemistry of paraffin-embedded human skeletal muscle tissue using CSB-PA006087ESR2HU at dilution of 1:100
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CSRP3 Antibody

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