Product Name	EFEMP1
Chinese Name	原纤维蛋白样蛋白DHRD抗体
Alias	DHRD; Doyne honeycomb retinal degeneration; DRAD; EFEMP 1; EFEMP1; EGF containing fibulin like extracellular matrix protein 1; EGF containing fibulin like extracellular matrix protein 1 precursor; EGF-containing fibulin-like extracellular matrix protein 1; Epidermal growth factor containing fibulin like extracellular matrix protein 1; Extracellular protein S1 5; Extracellular protein S1-5; FBLN 3; FBLN3; FBLN3_HUMAN; FBNL; FIBL 3; FIBL-3; FIBL3; Fibrillin like; Fibrillin like protein; Fibrillin-like protein; FIBULIN 3; Fibulin-3; Fibulin3; FLJ35535; Heat shock 70 KD protein 1; MGC111353; MLVT; MTLV; S1 5; T16 protein.
Research Area	Tumour  Neurobiology  Signal transduction  Extracellular matrix
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Mouse,  (predicted: Human, Rat, )
Applications	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	53kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human EFEMP1: 401-493/493
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	This gene encodes a member of the fibulin family of extracellular matrix glycoproteins. Like all members of this family, the encoded protein contains tandemly repeated epidermal growth factor-like repeats followed by a C-terminus fibulin-type domain. This gene is upregulated in malignant gliomas and may play a role in the aggressive nature of these tumors. Mutations in this gene are associated with Doyne honeycomb retinal dystrophy. Alternatively spliced transcript variants that encode the same protein have been described.[provided by RefSeq, Nov 2009] Function: Binds EGFR, the EGF receptor, inducing EGFR autophosphorylation and the activation of downstream signaling pathways. May play a role in cell adhesion and migration. May function as a negative regulator of chondrocyte differentiation. In the olfactory epithelium, it may regulate glial cell migration, differentiation and the ability of glial cells to support neuronal neurite outgrowth. Subcellular Location: Secreted > extracellular space. Secreted > extracellular space > extracellular matrix. Localizes to the lamina propria underneath the olfactory epithelium. Tissue Specificity: In the eye, associated with photoreceptor outer and inner segment regions, the nerve fiber layer, outer nuclear layer and inner and outer plexiform layers of the retina. DISEASE: Defects in EFEMP1 are a cause of Doyne honeycomb retinal dystrophy (DHRD) [MIM:126600]; also known as malattia leventinese (MLVT) (ML). DHRD is an autosomal dominant disease characterized by yellow-white deposits known as drusen that accumulate beneath the retinal pigment epithelium. Similarity: Belongs to the fibulin family. Contains 6 EGF-like domains. SWISS: Q12805 Gene ID: 2202 Database links: Entrez Gene: 2202 Human Entrez Gene: 216616 Mouse Entrez Gene: 305604 Rat Omim: 601548 Human SwissProt: Q12805 Human SwissProt: Q8BPB5 Mouse SwissProt: O35568 Rat Unigene: 76224 Human Unigene: 44176 Mouse Unigene: 163265 Rat
Product Picture	Protein: lung(mouse) lysate at 40ug; Primary: rabbit Anti-EFEMP1 (SL14516R) at 1:300; Secondary: HRP conjugated Goat-Anti-rabbit IgG(SL0295G-HRP) at 1: 5000; Predicted band size: 53 kD Observed band size: 49 kD

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