Anti-Fibulin-5 Antibody (CAB9961)

Antibody Name:	Anti-Fibulin-5 Antibody
Antibody SKU:	CAB9961
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF
Reactivity:	Human, Mouse
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 199-448 of human FBLN5 (NP_006320.2).

Application:	WB IF
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:50 - 1:100
Reactivity:	Human, Mouse
Positive Samples:	SKOV3, HT-29, 293T, A-549, A375, Mouse kidney

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 199-448 of human FBLN5 (NP_006320.2).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	NEDG RSCQ DVNE CATE NPCV QTCV NTYG SFIC RCDP GYEL EEDG VHCS DMDE CSFS EFLC QHEC VNQP GTYF CSCP PGYI LLDD NRSC QDIN ECEH RNHT CNLQ QTCY NLQG GFKC IDPI RCEE PYLR ISDN RCMC PAEN PGCR DQPF TILY RDMD VVSG RSVP ADIF QMQA TTRY PGAY YIFQ IKSG NEGR EFYM RQTG PISA TLVM TRPI KGPR EIQL DLEM ITVN TVIN FRGS SVIR LRIY VSQY PF
Gene ID:	10516
Uniprot:	Q9UBX5
Cellular Location:	Secreted, extracellular matrix, extracellular space
Calculated MW:	50kDa
Observed MW:	72kDa

Synonyms:

FBLN5, ADCL2, ARCL1A, ARMD3, DANCE, EVEC, FIBL-5, HNARMD, UP50, fibulin-5

Background:

The protein encoded by this gene is a secreted, extracellular matrix protein containing an Arg-Gly-Asp (RGD) motif and calcium-binding EGF-like domains. It promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. It is prominently expressed in developing arteries but less so in adult vessels. However, its expression is reinduced in balloon-injured vessels and atherosclerotic lesions, notably in intimal vascular smooth muscle cells and endothelial cells. Therefore, the protein encoded by this gene may play a role in vascular development and remodeling. Defects in this gene are a cause of autosomal dominant cutis laxa, autosomal recessive cutis laxa type I (CL type I), and age-related macular degeneration type 3 (ARMD3).

UniProt Protein Function:	FBLN5: Promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. Could be a vascular ligand for integrin receptors and may play a role in vascular development and remodeling. Defects in FBLN5 are the cause of cutis laxa, autosomal dominant, type 2 (ADCL2). 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 FBLN5 are a cause of cutis laxa, autosomal recessive, type 1A (ARCL1A). 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. The clinical spectrum of autosomal recessive cutis laxa is highly heterogeneous with respect to organ involvement and severity. Type I autosomal recessive cutis laxa is a specific, life-threatening disorder with organ involvement, lung atelectasis and emphysema, diverticula of the gastrointestinal and genitourinary systems, and vascular anomalies. Associated cranial anomalies, late closure of the fontanel, joint laxity, hip dislocation, and inguinal hernia have been observed but are uncommon. Defects in FBLN5 are the cause of age-related macular degeneration type 3 (ARMD3). ARMD is a multifactorial disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid (known as drusen) that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Belongs to the fibulin family.
UniProt Protein Details:	Protein type:Secreted; Secreted, signal peptide Chromosomal Location of Human Ortholog: 14q32.1 Cellular Component: extracellular matrix; extracellular region; extracellular space; proteinaceous extracellular matrix Molecular Function:calcium ion binding; integrin binding; protein binding; protein C-terminus binding; protein homodimerization activity Biological Process: cell-matrix adhesion; elastic fiber assembly; extracellular matrix organization and biogenesis; regulation of cell growth; secretion Disease: Cutis Laxa, Autosomal Dominant 2; Cutis Laxa, Autosomal Recessive, Type Ia; Macular Degeneration, Age-related, 3
NCBI Summary:	The protein encoded by this gene is a secreted, extracellular matrix protein containing an Arg-Gly-Asp (RGD) motif and calcium-binding EGF-like domains. It promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. It is prominently expressed in developing arteries but less so in adult vessels. However, its expression is reinduced in balloon-injured vessels and atherosclerotic lesions, notably in intimal vascular smooth muscle cells and endothelial cells. Therefore, the protein encoded by this gene may play a role in vascular development and remodeling. Defects in this gene are a cause of autosomal dominant cutis laxa, autosomal recessive cutis laxa type I (CL type I), and age-related macular degeneration type 3 (ARMD3). [provided by RefSeq, Jul 2008]
UniProt Code:	Q9UBX5
NCBI GenInfo Identifier:	12643876
NCBI Gene ID:	10516
NCBI Accession:	Q9UBX5.1
UniProt Secondary Accession:	Q9UBX5,O75966, Q6IAL4, Q6UWA3,
UniProt Related Accession:	Q9UBX5
Molecular Weight:	50,180 Da
NCBI Full Name:	Fibulin-5
NCBI Synonym Full Names:	fibulin 5
NCBI Official Symbol:	FBLN5
NCBI Official Synonym Symbols:	EVEC; UP50; ADCL2; ARMD3; DANCE; ARCL1A; FIBL-5; HNARMD
NCBI Protein Information:	fibulin-5
UniProt Protein Name:	Fibulin-5
UniProt Synonym Protein Names:	Developmental arteries and neural crest EGF-like protein; Dance; Urine p50 protein; UP50
Protein Family:	Fibulin
UniProt Gene Name:	FBLN5
UniProt Entry Name:	FBLN5_HUMAN

	Western blot analysis of extracts of various cell lines, using FBLN5 antibody (CAB9961) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 60s.
	Immunofluorescence analysis of L929 cells using FBLN5 Polyclonal Antibody (CAB9961) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.

Antibodies	ELISA Kits
FBLN5 Antibody (PACO06485)	Human FBLN5 / Fibulin-5 ELISA Kit
FBLN5 Antibody (PACO09243)	Human FBLN5 (Fibulin 5) CLIA Kit (HUES00721)
	Human FBLN5 (Fibulin 5) ELISA Kit (HUES02322)
	Human Fibulin-5 (FBLN5) ELISA Kit

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)