catalog number :
MBS2025651
products full name :
Monoclonal Antibody to Elastin (ELN)
products short name :
[Elastin]
other names :
[elastin isoform a; Elastin; elastin; elastin; Tropoelastin]
products gene name :
[ELN]
other gene names :
[ELN; ELN; WS; WBS; SVAS; ADCL1]
purity :
Protein A + Protein G affinity chromatography
form :
Supplied as solution form in 0.01M PBS, pH7.4, containing 0.05% Proclin-300, 50% glycerol.
storage stability :
Storage: Avoid repeated freeze/thaw cycles. Store at 4ºC for frequent use. Aliquot and store at -20ºC for 24 months. Stability Test: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.
tested application :
Western Blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunoprecipitation (IP).
app notes :
Western blotting: 0.5-2ug/mL;. Immunohistochemistry: 5-20ug/mL;. Immunocytochemistry: 5-20ug/mL;. Optimal working dilutions must be determined by end user.
image1 heading :
Western Blot (WB)
image2 heading :
Western Blot (WB)
image3 heading :
Immunohistochemistry (IHC)
other info1 :
Organism Species: Homo sapiens (Human). Source: Monoclonal antibody preparation. Traits: Liquid. Immunogen: Recombinant ELN (Gly392~Ala645) expressed in E.coli
other info2 :
Conjugated Antibody: The APC conjugated antibody version of this item is also available as catalog #MBS2054061
ncbi acc num :
NP_000492.2
ncbi gb acc num :
NM_000501.3
ncbi pathways :
Elastic Fibre Formation Pathway (730310); Extracellular Matrix Organization Pathway (576262); Protein Digestion And Absorption Pathway (172847); Protein Digestion And Absorption Pathway (171868)
ncbi summary :
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. [provided by RefSeq, Aug 2017]
uniprot summary :
elastin: 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. Defects in ELN are the cause of cutis laxa, autosomal dominant, type 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. Defects in ELN are the cause of supravalvular aortic stenosis (SVAS). SVAS is a 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. Belongs to the elastin family. 13 isoforms of the human protein are produced by alternative splicing. Protein type: Extracellular matrix; Secreted; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 7q11.23. Cellular Component: extracellular matrix; extracellular region; proteinaceous extracellular matrix. Molecular Function: extracellular matrix structural constituent; protein binding. Biological Process: blood circulation; cell proliferation; extracellular matrix disassembly; extracellular matrix organization; organ morphogenesis; respiratory gaseous exchange. Disease: Cutis Laxa, Autosomal Dominant 1; Supravalvular Aortic Stenosis; Williams-beuren Syndrome