Elastin [BA-4]

ArtNr	20-272-193019
Hersteller	GENWAY
Menge	0.5 ml
Kategorie	Oncology Antibodies Cell Biology Immunohistochemistry Immunoprecipitation Western Blot Cell Stainings ECM Primary Antibodies By Organ Lung Cancer
Typ	Antibody
Applikationen	WB, IP, IHC
Clon	BA-4
Specific against	other
ECLASS 10.1	32160702
ECLASS 11.0	32160702
UNSPSC	12352203
Alias	GWB-399EC6
Similar products	20-272-193019
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Genway ID:	GWB-399EC6
Clone:	BA-4
Isotype:	IgG1
Immunogen:	Bovine alpha-Elastin.
Antigen Species:	Bovine
Specificity:	This antibody recognizes insoluble elastin alpha-elastin soluble non-cross linked precursor of elastin (tropoelastin).
Positive Control:	Heart
Target:	Elastin
Localization:	Connective tissue
Concentration:	Ascites Storage
Buffer:	Contains sodium azide
Application Note:	This antibody may be diluted to a titer of 1:50-1:100 in an ABC method. We suggest an incubation period of 30 minutes at RT. Proteolytic treatment prior to immunostaining is required for formailn fixed paraffin embedded tissue. For WB: Use at a dilution of 1:100 - 1:500. Not tested in other applications. Optimal dilutions/concentrations should be determined by the researcher. Elastin is an important polymeric protein of connective tissue that imparts elasticity to vertebrate elastic tissues.
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 (By similarity).
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 & gt; magnesium & gt; 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.
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.
Ptm:	Hydroxylation on proline residues within the sequence motif GXPG is most likely 4-hydroxy as this fits the requirement for 4-hydroxylation in vertebrates (By similarity).
Disease:	Defects in ELN are a cause of autosomal dominant cutis laxa [MIM:123700]. Cutis laxa is a rare connective tissue disorder characterized by loose hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. The skin changes are often accompanied by extracutaneous manifestations including pulmonary emphysema bladder diverticula pulmonary artery stenosis and pyloric stenosis.
Disease:	Haploinsufficiency of ELN may be the cause of certain cardiovascular and musculo-skeletal abnormalities observed in Williams-Beuren syndrome (WBS) [MIM:194050]. WBS is a rare developmental disorder and a contiguous gene deletion syndrome involving genes from chromosome band 7q11. 23.
Disease:	Defects in ELN are the cause of supravalvular aortic stenosis (SVAS) [MIM:185500]. 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.
Similarity:	Belongs to the elastin family.

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