Rabbit ELN Polyclonal Antibody | anti-ELN antibody

ELN Polyclonal Antibody

Cat. Num. AAA2520825

• Gene Names: ELN; WS; WBS; SVAS
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: Antigen affinity purification
• Synonyms: ELN; Polyclonal Antibody; ELN Polyclonal Antibody; WS; WBS; SVAS; anti-ELN antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Antigen affinity purification
• Concentration: 2.1mg/mL (varies by lot)
• Sequence Length: 658

• Applicable Applications for anti-ELN antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1:200-1:1000; IHC: 1:50-1:200
• Immunogen: Recombinant protein of human ELN
• Buffer: PBS with 0.05% sodium azide, 50% glycerol, pH7.3
• Preparation and Storage: Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.

Western Blot (WB)

(Western Blot analysis of Human hepatocellular carcinoma tissue using ELN Polyclonal Antibody at dilution of 1:500)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded Human ovarian cancer using ELN Polyclonal Antibody at dilution of 1:50)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded Human colon cancer using ELN Polyclonal Antibody at dilution of 1:50)

Related Product Information for anti-ELN antibody

This gene encodes a protein that is one of the two components of elastic fibers. 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. Deletions and mutations in this gene are associated with supravalvular aortic stenosis (SVAS) and autosomal dominant cutis laxa. Multiple transcript variants encoding different isoforms have been found for this gene.

Product Categories/Family for anti-ELN antibody

Cancer

NCBI and Uniprot Product Information

• NCBI GI #: 41388841
• NCBI GeneID: 2006
• Molecular Weight: 60,980 Da
• NCBI Official Full Name: ELN protein
• NCBI Official Synonym Full Names: elastin
• NCBI Official Symbol: ELN
• NCBI Official Synonym Symbols: WS; WBS; SVAS
• NCBI Protein Information: elastin; tropoelastin
• UniProt Protein Name: Elastin
• Protein Family: Elastin
• UniProt Gene Name: ELN
• UniProt Entry Name: ELN_HUMAN

NCBI Description

This gene encodes a protein that is one of the two components of elastic fibers. 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. Deletions and mutations in this gene are associated with supravalvular aortic stenosis (SVAS) and autosomal dominant cutis laxa. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

Uniprot Description

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: Secreted; Extracellular matrix; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 7q11.23

Cellular Component: proteinaceous extracellular matrix; extracellular region

Molecular Function: protein binding; extracellular matrix structural constituent

Biological Process: extracellular matrix disassembly; cell proliferation; extracellular matrix organization and biogenesis; organ morphogenesis; blood circulation; respiratory gaseous exchange

Disease: Cutis Laxa, Autosomal Dominant 1; Williams-beuren Syndrome; Supravalvular Aortic Stenosis