Anti-EFEMP2 항체 (CAB10018)

Antibody Name:	Anti-EFEMP2 Antibody
Antibody SKU:	CAB10018
Antibody Size:	20uL, 50uL, 100uL
Application:	WB
Reactivity:	Human
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 26-250 of human EFEMP2 (NP_058634.4).

Application:	WB
Recommended Dilution:	WB 1:500 - 1:1000
Reactivity:	Human
Positive Samples:	SH-SY5Y, HT-1080

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 26-250 of human EFEMP2 (NP_058634.4).
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:	SPQD SEEP DSYT ECTD GYEW DPDS QHCR DVNE CLTI PEAC KGEM KCIN HYGG YLCL PRSA AVIN DLHG EGPP PPVP PAQH PNPC PPGY EPDD QDSC VDVD ECAQ ALHD CRPS QDCH NLPG SYQC TCPD GYRK IGPE CVDI DECR YRYC QHRC VNLP GSFR CQCE PGFQ LGPN NRSC VDVN ECDM GAPC EQRC FNSY GTFL CRCH QGYE LHRD GFSC SDID ECSY S
Gene ID:	30008
Uniprot:	O95967
Cellular Location:	Secreted
Calculated MW:	49kDa
Observed MW:	55kDa

Synonyms:

EFEMP2, ARCL1B, FBLN4, MBP1, UPH1

Background:

A large number of extracellular matrix proteins have been found to contain variations of the epidermal growth factor (EGF) domain and have been implicated in functions as diverse as blood coagulation, activation of complement and determination of cell fate during development. The protein encoded by this gene contains four EGF2 domains and six calcium-binding EGF2 domains. This gene is necessary for elastic fiber formation and connective tissue development. Defects in this gene are cause of an autosomal recessive cutis laxa syndrome. Alternatively spliced transcript variants have been identified for this gene.

UniProt Protein Function:	EFEMP2: Defects in EFEMP2 are the cause of cutis laxa, autosomal recessive, type 1B (ARCL1B). A connective tissue disorder characterized by 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. ARCL1B features include emphysema, lethal pulmonary artery occlusion, aortic aneurysm, cardiopulmonary insufficiency, birth fractures, arachnodactyly, and fragility of blood vessels. Belongs to the fibulin family.
UniProt Protein Details:	Protein type:Secreted, signal peptide; Secreted Chromosomal Location of Human Ortholog: 11q13.1 Cellular Component: basement membrane; extracellular region Molecular Function:extracellular matrix structural constituent; protein binding Disease: Cutis Laxa, Autosomal Recessive, Type Ib
NCBI Summary:	A large number of extracellular matrix proteins have been found to contain variations of the epidermal growth factor (EGF) domain and have been implicated in functions as diverse as blood coagulation, activation of complement and determination of cell fate during development. The protein encoded by this gene contains four EGF2 domains and six calcium-binding EGF2 domains. This gene is necessary for elastic fiber formation and connective tissue development. Defects in this gene are cause of an autosomal recessive cutis laxa syndrome. Alternatively spliced transcript variants have been identified for this gene. [provided by RefSeq, Jan 2011]
UniProt Code:	O95967
NCBI GenInfo Identifier:	134047775
NCBI Gene ID:	30008
NCBI Accession:	O95967.3
UniProt Secondary Accession:	O95967,O75967, A8K7R4, B3KM31, B3KQT1,
UniProt Related Accession:	O95967
Molecular Weight:	49,405 Da
NCBI Full Name:	EGF-containing fibulin-like extracellular matrix protein 2
NCBI Synonym Full Names:	EGF containing fibulin like extracellular matrix protein 2
NCBI Official Symbol:	EFEMP2
NCBI Official Synonym Symbols:	MBP1; UPH1; FBLN4; ARCL1B
NCBI Protein Information:	EGF-containing fibulin-like extracellular matrix protein 2
UniProt Protein Name:	EGF-containing fibulin-like extracellular matrix protein 2
UniProt Synonym Protein Names:	Fibulin-4; FIBL-4; Protein UPH1
Protein Family:	EGF-containing fibulin-like extracellular matrix protein
UniProt Gene Name:	EFEMP2
UniProt Entry Name:	FBLN4_HUMAN

Western blot analysis of extracts of various cell lines, using EFEMP2 antibody (CAB10018) 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 Enhanced Kit (RM00021). Exposure time: 90s.

Antibodies	ELISA Kits
EFEMP2 Antibody (PACO04021)	Human FBLN4 / Fibulin 4 ELISA Kit
EFEMP2 Antibody (PACO09006)	Human FBLN4 (Fibulin 4) CLIA Kit (HUES00270)
	Human FBLN4 (Fibulin 4) ELISA Kit (HUES01593)

Secondary Antibody

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