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Mouse Anti-ELN (AA 665-673) Recombinant Antibody (CBFYE-0798) (CBMAB-E1245-FY)

This product is mouse antibody that recognizes ELN. The antibody CBFYE-0798 can be used for immunoassay techniques such as: ELISA, IF, IP, WB.
See all ELN antibodies

Summary

Host Animal
Mouse
Specificity
Cattle, Chicken, Human, Mouse
Clone
CBFYE-0798
Antibody Isotype
IgG
Application
ELISA, IF, IP, WB

Basic Information

Immunogen
Tropoelastin from 17-day old lathyric chick embryo aortae, extracted and purified according to the method of Rich and Foster, Meth. Enzymol.
Specificity
Cattle, Chicken, Human, Mouse
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.6
Preservative
0.09% Sodium azide
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
AA 665-673

Target

Full Name
Elastin
Introduction
This gene encodes a protein that is one of the two components of elastic fibers. Elastic fibers comprise part of the extracellular matrix and confer elasticity to organs and tissues including the heart, skin, lungs, ligaments, and blood vessels. The encoded protein is rich in hydrophobic amino acids such as glycine and proline, which form mobile hydrophobic regions bounded by crosslinks between lysine residues. Degradation products of the encoded protein, known as elastin-derived peptides or elastokines, bind the elastin receptor complex and other receptors and stimulate migration and proliferation of monocytes and skin fibroblasts. Elastokines can also contribute to cancer progression. Deletions and mutations in this gene are associated with supravalvular aortic stenosis (SVAS) and autosomal dominant cutis laxa.
Entrez Gene ID
Human2006
Cattle280781
Mouse13717
Chicken396441
UniProt ID
HumanP15502
CattleP04985
MouseP54320
ChickenP07916
Alternative Names
Elastin; Tropoelastin; Supravalvular Aortic Stenosis; Williams-Beuren Syndrome; ADCL1; SVAS; WBS; WS
Research Area
Major structural protein of tissues such as aorta and nuchal ligament, which must expand rapidly and recover completely. Molecular determinant of the late arterial morphogenesis, stabilizing arterial structure by regulating proliferation and organization of vascular smooth muscle (By similarity).
Biological Process
Animal organ morphogenesis Source: ProtInc
Aortic valve morphogenesis Source: BHF-UCL
Blood circulation Source: ProtInc
Extracellular matrix organization Source: Ensembl
Outflow tract morphogenesis Source: BHF-UCL
Regulation of actin filament polymerization Source: Ensembl
Regulation of smooth muscle cell proliferation Source: GO_Central
Respiratory gaseous exchange by respiratory system Source: ProtInc
Skeletal muscle tissue development Source: Ensembl
Stress fiber assembly Source: Ensembl
Cellular Location
Extracellular matrix. Extracellular matrix of elastic fibers.
Involvement in disease
Cutis laxa, autosomal dominant, 1 (ADCL1):
A connective tissue disorder characterized by loose, hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. Face, hands, feet, joints, and torso may be differentially affected. Additional variable clinical features are gastrointestinal diverticula, hernia, and genital prolapse. Rare manifestations are pulmonary artery stenosis, aortic aneurysm, bronchiectasis, and emphysema.
Supravalvular aortic stenosis (SVAS):
Congenital narrowing of the ascending aorta which can occur sporadically, as an autosomal dominant condition, or as one component of Williams-Beuren syndrome.
ELN is located in the Williams-Beuren syndrome (WBS) critical region. WBS results from a hemizygous deletion of several genes on chromosome 7q11.23, thought to arise as a consequence of unequal crossing over between highly homologous low-copy repeat sequences flanking the deleted region. Haploinsufficiency of ELN may be the cause of certain cardiovascular and musculo-skeletal abnormalities observed in the disease (PubMed:8812460).
PTM
Elastin is formed through the cross-linking of its soluble precursor tropoelastin. Cross-linking is initiated through the action of lysyl oxidase on exposed lysines to form allysine. Subsequent spontaneous condensation reactions with other allysine or unmodified lysine residues result in various bi-, tri-, and tetrafunctional cross-links. The most abundant cross-links in mature elastin fibers are lysinonorleucine, allysine aldol, desmosine, and isodesmosine.
Hydroxylation on proline residues within the sequence motif, GXPG, is most likely 4-hydroxy as this fits the requirement for 4-hydroxylation in vertebrates.

Lin, C. J., Cocciolone, A. J., & Wagenseil, J. E. (2022). Elastin, arterial mechanics, and stenosis. American Journal of Physiology-Cell Physiology, 322(5), C875-C886.

Procknow, S. S., & Kozel, B. A. (2022). Emerging mechanisms of elastin transcriptional regulation. American Journal of Physiology-Cell Physiology, 323(3), C666-C677.

Kim, J., Cocciolone, A. J., Staiculescu, M. C., Mecham, R. P., & Wagenseil, J. E. (2022). Passive biaxial mechanical behavior of newborn mouse aorta with and without elastin. Journal of the Mechanical Behavior of Biomedical Materials, 126, 105021.

Peng, X., Guo, Z., Zhang, Y., Sun, B., & Zhang, Q. (2022). EFEMP1 in direct inguinal hernia: correlation with TIMP3 and regulation toward elastin homoeostasis as well as fibroblast mobility. Journal of Investigative Surgery, 35(1), 203-211.

Dave, J. M., Chakraborty, R., Ntokou, A., Saito, J., Saddouk, F. Z., Feng, Z., ... & Greif, D. M. (2022). JAGGED1/NOTCH3 activation promotes aortic hypermuscularization and stenosis in elastin deficiency. The Journal of clinical investigation, 132(5).

Lin, C. J., Hunkins, B. M., Roth, R. A., Lin, C. Y., Wagenseil, J. E., & Mecham, R. P. (2021). Vascular smooth muscle cell subpopulations and neointimal formation in mouse models of elastin insufficiency. Arteriosclerosis, thrombosis, and vascular biology, 41(12), 2890-2905.

Li, J., Xu, X., Jiang, Y., Hansbro, N. G., Hansbro, P. M., Xu, J., & Liu, G. (2020). Elastin is a key factor of tumor development in colorectal cancer. BMC cancer, 20(1), 1-12.

Kim, J., Cocciolone, A. J., Staiculescu, M. C., Mecham, R. P., & Wagenseil, J. E. (2020). Captopril treatment during development alleviates mechanically induced aortic remodeling in newborn elastin knockout mice. Biomechanics and modeling in mechanobiology, 19(1), 99-112.

Hayano, S., Okuno, Y., Tsutsumi, M., Inagaki, H., Fukasawa, Y., Kurahashi, H., ... & Kato, T. (2019). Frequent intragenic microdeletions of elastin in familial supravalvular aortic stenosis. International Journal of Cardiology, 274, 290-295.

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For research use only. Not intended for any clinical use.

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