Fibulin 3 (FBLN3)

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EFEMP1; DHRD; DRAD; FBNL; MLVT; MTLV; S1-5; EGF-Containing Fibulin-Like Extracellular Matrix Protein 1; Fibrillin-Like; Extracellular protein S1-5

Fibulin 3 (FBLN3)
EGF-containing fibulin-like extracellular matrix protein 1 spans approximately 18 kb of genomic DNA and consists of 12 exons. Alternative splice patterns in the 5' UTR result in three transcript variants encoding the same extracellular matrix protein. Mutations in this gene are associated with Doyne honeycomb retinal dystrophy. The cDNA for this gene, designated 'fibrillin-like' (FBNL), was isolated from a fibroblast cDNA library. The FBNL cDNA probe detected 2 transcripts of 2.2 and 3.0 kb in mRNA from multiple tissues. The FBNL gene is expressed in many tissues but it is not expressed in brain and lymphocytes. the amino acid sequence of the FBNL gene is 36.3% identical to FBN1 and 35.4% identical to FBN2 and that FBNL contains repeated cbEGF-like domains, a cardinal motif of FBN genes.

Organism species: Homo sapiens (Human)

CATALOG NO.PRODUCT NAMEAPPLICATIONS
ProteinsRPF422Hu01Recombinant Fibulin 3 (FBLN3)SDS-PAGE; WB; ELISA; IP.
RPF422Hu02Recombinant Fibulin 3 (FBLN3)SDS-PAGE; WB; ELISA; IP.
AntibodiesPAF422Hu01Polyclonal Antibody to Fibulin 3 (FBLN3)WB, ICC, IHC-P, IHC-F, ELISA
LAF422Hu71Biotin-Linked Polyclonal Antibody to Fibulin 3 (FBLN3)IHC;WB;ELISA
MAF422Hu21Monoclonal Antibody to Fibulin 3 (FBLN3)WB, ICC, IHC-P, IHC-F, ELISA
PAF422Hu02Polyclonal Antibody to Fibulin 3 (FBLN3)WB, ICC, IHC-P, IHC-F, ELISA
Assay KitsSEF422HuELISA Kit for Fibulin 3 (FBLN3)Enzyme-linked immunosorbent assay for Antigen Detection
SCF422HuCLIA Kit for Fibulin 3 (FBLN3)Chemiluminescent immunoassay for Antigen Detection

Organism species: Mus musculus (Mouse)

CATALOG NO.PRODUCT NAMEAPPLICATIONS
ProteinsRPF422Mu01Recombinant Fibulin 3 (FBLN3)SDS-PAGE; WB; ELISA; IP.
RPF422Mu02Recombinant Fibulin 3 (FBLN3)SDS-PAGE; WB; ELISA; IP.
AntibodiesPAF422Mu01Polyclonal Antibody to Fibulin 3 (FBLN3)WB, ICC, IHC-P, IHC-F, ELISA
PAF422Mu02Polyclonal Antibody to Fibulin 3 (FBLN3)WB, ICC, IHC-P, IHC-F, ELISA
Assay KitsSEF422MuELISA Kit for Fibulin 3 (FBLN3)Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rattus norvegicus (Rat)

CATALOG NO.PRODUCT NAMEAPPLICATIONS
ProteinsRPF422Ra01Recombinant Fibulin 3 (FBLN3)SDS-PAGE; WB; ELISA; IP.
AntibodiesPAF422Ra01Polyclonal Antibody to Fibulin 3 (FBLN3)WB, ICC, IHC-P, IHC-F, ELISA
Assay Kitsn/aCLIA Kit for Fibulin 3 (FBLN3)CLIA Kit Customized Service Offer
n/aELISA Kit for Fibulin 3 (FBLN3)ELISA Kit Customized Service Offer
  1. "An overexpressed gene transcript in senescent and quiescent human fibroblasts encoding a novel protein in the epidermal growth factor-like repeat family stimulates DNA synthesis."Mol. Cell. Biol. 15:120-128(1995) [PubMed] [Europe PMC] [Abstract]
  2. "Structure and chromosomal assignment of the human S1-5 gene (FBNL) that is highly homologous to fibrillin."Genomics 35:590-592(1996) [PubMed] [Europe PMC] [Abstract]
  3. "Sequence, recombinant expression and tissue localization of two novel extracellular matrix proteins, fibulin-3 and fibulin-4."Matrix Biol. 18:469-480(1999) [PubMed] [Europe PMC] [Abstract]
  4. "EFEMP1 is not associated with sporadic early onset drusen."Ophthalmic Genet. 22:27-34(2001) [PubMed] [Europe PMC] [Abstract]
  5. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  6. "Generation and annotation of the DNA sequences of human chromosomes 2 and 4." Nature 434:724-731(2005) [PubMed] [Europe PMC] [Abstract]
  7. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  8. "Aberrant accumulation of EFEMP1 underlies drusen formation in Malattia Leventinese and age-related macular degeneration."Proc. Natl. Acad. Sci. U.S.A. 99:13067-13072(2002) [PubMed] [Europe PMC] [Abstract]
  9. "Tissue inhibitor of metalloproteinases-3 (TIMP-3) is a binding partner of epithelial growth factor-containing fibulin-like extracellular matrix protein 1 (EFEMP1). Implications for macular degenerations."J. Biol. Chem. 279:30469-30473(2004) [PubMed] [Europe PMC] [Abstract]
  10. "EFEMP1 binds the EGF receptor and activates MAPK and Akt pathways in pancreatic carcinoma cells."Biol. Chem. 390:1293-1302(2009) [PubMed] [Europe PMC] [Abstract]
  11. "ECM1 interacts with fibulin-3 and the beta 3 chain of laminin 332 through its serum albumin subdomain-like 2 domain."Matrix Biol. 28:160-169(2009) [PubMed] [Europe PMC] [Abstract]
  12. "Fibulin-3 is uniquely upregulated in malignant gliomas and promotes tumor cell motility and invasion."Mol. Cancer Res. 7:1756-1770(2009) [PubMed] [Europe PMC] [Abstract]
  13. "Fibulin-3 negatively regulates chondrocyte differentiation."Biochem. Biophys. Res. Commun. 391:1116-1121(2010) [PubMed] [Europe PMC] [Abstract]
  14. "Resveratrol-induced changes of the human adipocyte secretion profile."J. Proteome Res. 11:4733-4743(2012) [PubMed] [Europe PMC] [Abstract]
  15. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  16. "A single EFEMP1 mutation associated with both malattia Leventinese and Doyne honeycomb retinal dystrophy."Nat. Genet. 22:199-202(1999) [PubMed] [Europe PMC] [Abstract]
  17. "Dominant radial drusen and Arg345Trp EFEMP1 mutation."Am. J. Ophthalmol. 131:810-812(2001) [PubMed] [Europe PMC] [Abstract]
  18. "The R345W mutation in EFEMP1 is pathogenic and causes AMD-like deposits in mice."Hum. Mol. Genet. 16:2411-2422(2007) [PubMed] [Europe PMC] [Abstract]