ALPHA ELASTIN


ArtNr	18-783-77565
Hersteller	GENWAY
Menge	1 ml
Kategorie	Oncology Antibodies Cell Biology Immunohistochemistry ELISA Cell Stainings ECM Primary Antibodies By Organ Lung Cancer
Typ	Antibody
Applikationen	IHC, ELISA
Specific against	other
ECLASS 10.1	32160702
ECLASS 11.0	32160702
UNSPSC	12352203
Alias	GWB-FD1FA2
Similar products	18-783-77565
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Genway ID:	GWB-FD1FA2
Specificity:	ALPHA ELASTIN
Specificity:	ALPHA ELASTIN
Immunogen:	Native
Specificity Note:	100% with beta elastin at antibody dilution of 1/6K. Cross reacts with alpha elastin from cow.
Immunohistology:	Fixation with 10% formalin is recommended.
Preparation:	Ig fraction prepared by ammonium sulphate precipitation
Buffer Solution:	Phosphate buffered saline
Preservative Stabilisers:		0. 09% Sodium Azide (NaN3)
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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