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Home / Primary Antibodies / IHC Antibodies

FBN1 Antibody

SKU : BHA10557726

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CUSABIO TECHONOLOGY LLC

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Mfr.No.	CSB-PA142599
Alternative Names	MASS; MFS1; OCTD; SGS; WMS
Categories	Primary Antibodies
Clonality	polyclonal
Description	Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-1-containing microfibrils provide long-term force bearing structural support. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively.Ehab El-Hallous, J. Biol. Chem., Mar 2007; 282: 8935 - 8946.Shazia S. Chaudhry, J. Cell Biol., Jan 2007; 176: 355 - 367.Kerstin Tiedemann, J. Biol. Chem., Sep 2001; 276: 36035 - 36042.Liliana Schaefer, Am. J. Pathol., Aug 2004; 165: 383 - 396.
Host	Rabbit
Immunogen	Synthesized peptide derived from Human Fibrillin-1.
Involvement In Disease	Marfan syndrome (MFS); Ectopia lentis 1, isolated, autosomal dominant (ECTOL1); Weill-Marchesani syndrome 2 (WMS2); Overlap connective tissue disease (OCTD); Stiff skin syndrome (SSKS); Geleophysic dysplasia 2 (GPHYSD2); Acromicric dysplasia (ACMICD); Marfan lipodystrophy syndrome (MFLS)
Raised In	Rabbit
Reactivity	Human
Regulatory	RUO
Relevance	Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-1-containing microfibrils provide long-term force bearing structural support. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. Ehab El-Hallous, J. Biol. Chem., Mar 2007; 282: 8935 - 8946. Shazia S. Chaudhry, J. Cell Biol., Jan 2007; 176: 355 - 367. Kerstin Tiedemann, J. Biol. Chem., Sep 2001; 276: 36035 - 36042. Liliana Schaefer, Am. J. Pathol., Aug 2004; 165: 383 - 396.
Species	Homo Sapiens (Human)
Specificity	The antibody detects endogenous levels of total Fibrillin-1 protein.
Subcellular Location	Secreted, Note=Fibrillin-1 and Asprosin chains are still linked together during the secretion from cells, but are subsequently separated by furin (PubMed:24982166), SUBCELLULAR LOCATION: Asprosin: Secreted

Uniprot	P35555
Gene Id	2200
Database Links	HGNC:3603 OMIM:102370 KEGG:hsa:2200 STRING:9606.ENSP00000325527 UniGene:Hs.591133
Function	Fibrillin-1
Protein Families	Fibrillin family
Weight	312,237 Da

Buffer	Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Form	Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Format	liquid
Purification	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Purity	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Storage	Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Applications

ELISA, IHC

Description

ELISA ; IHC, IHC ; ELISA

Dilution

IHC	1:50-1:100

Tested Application

ELISA,IHC;IHC:1:50-1:100

Datasheet
ELISA Protocol
Immunohistochemistry (IHC) Protocol

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SKU	Supplier No.	Size	Your price	Quantity
BHA10557726	CSB-PA142599	100ul	$297		Add to Cart

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