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AB171952

Anti-Ellis van Creveld syndrome antibody [EPR10408]

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Rabbit Recombinant Monoclonal Ellis van Creveld syndrome antibody. Suitable for WB, ICC/IF and reacts with Human samples.

View Alternative Names

EvC complex member EVC, DWF-1, Ellis-van Creveld syndrome protein, EVC

2 Images
Western blot - Anti-Ellis van Creveld syndrome antibody [EPR10408] (AB171952)
  • WB

Supplier Data

Western blot - Anti-Ellis van Creveld syndrome antibody [EPR10408] (AB171952)

All lanes:

Western blot - Anti-Ellis van Creveld syndrome antibody [EPR10408] (ab171952) at 1/1000 dilution

Lane 1:

MCF7 cell lysate at 10 µg

Lane 2:

K562 cell lysate at 10 µg

Lane 3:

HeLa cell lysate at 10 µg

Lane 4:

293T cell lysate at 10 µg

Predicted band size: 112 kDa

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Immunocytochemistry/ Immunofluorescence - Anti-Ellis van Creveld syndrome antibody [EPR10408] (AB171952)
  • ICC/IF

Supplier Data

Immunocytochemistry/ Immunofluorescence - Anti-Ellis van Creveld syndrome antibody [EPR10408] (AB171952)

Immunofluorescent analysis of HeLa cells labeling Ellis van Creveld syndrome with ab171952 at 1/50 dilution (red). DAPI nuclear staining (blue).

Key facts

Host species

Rabbit

Clonality

Monoclonal

Clone number

EPR10408

Isotype

IgG

Carrier free

No

Reacts with

Human

Applications

WB, ICC/IF

applications

Immunogen

The exact immunogen used to generate this antibody is proprietary information.

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "IP" : {"fullname" : "Immunoprecipitation", "shortname":"IP"}, "FlowCyt" : {"fullname" : "Flow Cytometry", "shortname":"Flow Cyt"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"}, "ICCIF" : {"fullname" : "Immunocytochemistry/ Immunofluorescence", "shortname":"ICC/IF"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "IP-species-checked": "notRecommended", "IP-species-dilution-info": "", "IP-species-notes": "<p></p>", "FlowCyt-species-checked": "notRecommended", "FlowCyt-species-dilution-info": "", "FlowCyt-species-notes": "<p></p>", "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/1000 - 1/5000", "WB-species-notes": "<p></p>", "IHCP-species-checked": "notRecommended", "IHCP-species-dilution-info": "", "IHCP-species-notes": "<p></p>", "ICCIF-species-checked": "testedAndGuaranteed", "ICCIF-species-dilution-info": "1/50 - 1/100", "ICCIF-species-notes": "<p></p>" }, "Mouse": { "IP-species-checked": "notRecommended", "IP-species-dilution-info": "", "IP-species-notes": "", "FlowCyt-species-checked": "notRecommended", "FlowCyt-species-dilution-info": "", "FlowCyt-species-notes": "", "WB-species-checked": "predicted", "WB-species-dilution-info": "", "WB-species-notes": "", "IHCP-species-checked": "notRecommended", "IHCP-species-dilution-info": "", "IHCP-species-notes": "", "ICCIF-species-checked": "predicted", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "" }, "Rat": { "IP-species-checked": "notRecommended", "IP-species-dilution-info": "", "IP-species-notes": "", "FlowCyt-species-checked": "notRecommended", "FlowCyt-species-dilution-info": "", "FlowCyt-species-notes": "", "WB-species-checked": "predicted", "WB-species-dilution-info": "", "WB-species-notes": "", "IHCP-species-checked": "notRecommended", "IHCP-species-dilution-info": "", "IHCP-species-notes": "", "ICCIF-species-checked": "predicted", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "" } } }
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Product details

Patented technology
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.

What are the advantages of a recombinant monoclonal antibody?
This product is a recombinant monoclonal antibody, which offers several advantages including:

  • - High batch-to-batch consistency and reproducibility
  • - Improved sensitivity and specificity
  • - Long-term security of supply
  • - Animal-free batch production

For more information, read more on recombinant antibodies.

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Properties and storage information

Form
Liquid
Purification technique
Affinity purification Protein A
Storage buffer
pH: 7.2 - 7.4 Constituents: 59% PBS, 40% Glycerol (glycerin, glycerine), 0.35% Sodium citrate, 0.17% Sodium chloride, 0.05% BSA, 0.03% EDTA
Shipped at conditions
Blue Ice
Appropriate short-term storage duration
1-2 weeks
Appropriate short-term storage conditions
+4°C
Appropriate long-term storage conditions
-20°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

Ellis-van Creveld syndrome (EVC syndrome) is a rare genetic disorder also known as chondroectodermal dysplasia. The condition arises due to mutations in the EVC and EVC2 genes which encode proteins involved in the Hedgehog signaling pathway. These proteins function together as part of a multiprotein complex that affects the transport of Gli transcription factors. Structurally EVC proteins are not separately described with specific molecular masses because EVC functions in concert with other components. These proteins show expression primarily in tissues related to bone and tooth development including cartilaginous and ectodermal tissues.
Biological function summary

EVC and EVC2 proteins interact with other members of the Hedgehog signaling pathway orchestrating the development of skeletal elements and digits. They form part of the protein complex within the primary cilia which are microtubule-based organelles essential for signal transduction in developmental pathways. Proper functioning of this complex regulates the activity and movement of Gli transcription factors which are pivotal in gene expression for cell differentiation and growth during embryogenesis.

Pathways

Ellis-van Creveld syndrome is deeply linked with the Hedgehog signaling pathway important for embryonic development and morphogenesis. This pathway includes proteins such as Smoothened (SMO) and Patched (PTCH1) which interact upstream of EVC proteins to modulate signaling strength and duration. Due to its role in the Hedgehog pathway alteration in EVC function may disrupt normal signaling activities leading to developmental anomalies.

Ellis-van Creveld syndrome manifests in symptoms such as skeletal dysplasia and polydactyly with its roots in the malfunction of Hedgehog pathway signaling. This disorder shows a distinct connection to diseases involving ciliopathies where other ciliary proteins like IFT proteins might show related dysfunctions. The genetic basis involving EVC and EVC2 provides an important link to understanding the pathogenesis of these structural and developmental conditions.
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Product protocols

For this product, it's our understanding that no specific protocols are required. You can visit:
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Target data

Component of the EvC complex that positively regulates ciliary Hedgehog (Hh) signaling. Involved in endochondral growth and skeletal development.
See full target information Ellis-van Creveld syndrome protein
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Product promise

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For full details, please see our Terms & Conditions

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