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Rabbit Anti-alpha Elastin/Tropoelastin/FITC Conjugated antibody
background:
Elastin is a major structural protein of tissues such as aorta, which must expand rapidly and recover completely. Elastin chains are cross-linked together into an extensible 3D network. It is a molecular determinant of late arterial morphogenesis, stabilizing arterial structure by regulating proliferation and organization of vascular smooth muscle. Elastin forms a complex with BGN and MFAP2.
Function:
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.
Subunit:
The polymeric elastin chains are cross-linked together into an extensible 3D network. Forms a ternary complex with BGN and MFAP2. Interacts with MFAP2 via divalent cations (calcium > magnesium > manganese) in a dose-dependent and saturating manner.
Subcellular Location:
Secreted, extracellular space, extracellular matrix. Note=Extracellular matrix of elastic fibers.
Tissue Specificity:
Expressed within the outer myometrial smooth muscle and throughout the arteriolar tree of uterus (at protein level). Also expressed in the large arteries, lung and skin.
DISEASE:
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.
Similarity:
Belongs to the elastin family.
Database links:
Entrez Gene: 2006 Human
Entrez Gene: 13717 Mouse
Omim: 130160 Human
SwissProt: P15502 Human
SwissProt: P5464 Mouse
Unigene: 647061 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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