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Fibrillin 1 (FBN1)

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FBN; MASS; MFS1; OCTD; SGS

Fibrillin 1 (FBN1)
CCC A593
HGNC 3603
MGI 95489
PubMed 8541880
RGD 620908
UniProt P35555
Wiki FBN1

Fibrillin-1 is a major component of the microfibrils that form a sheath surrounding the amorphous elastin. It is believed that the microfibrils are composed of end-to-end polymers of fibrillin. To date, 3 forms of fibrillin have been described. The fibrillin-1 protein was isolated by Engvall in 1986, and mutations in the gene have been linked to the Marfan syndrome. At present more than 100 different mutations have been described. Trask et al. (1999) found that human FIB1 and FIB2 homodimerized via an N-terminal region and that the interaction was stabilized by disulphide bonds. Dimer formation occurred intracellularly, suggesting that the process of fibrillin aggregation initiates early after biosynthesis of the molecules.

Asprosin is generated from the gene FBN1 (amino acid residues 2732–2871), which also generates fibrillin.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA593Hu01 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
RPA593Hu02 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
RPA593Hu05 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
RPA593Hu03 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
RPA593Hu04 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
RPA593Hu06 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
RPA593Hu07 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA593Hu01 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC; IP.
MAA593Hu22 Monoclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC; IP.
PAA593Hu02 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; ICC; IHC-P; IHC-F; ELISA.
LAA593Hu81 FITC-Linked Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC; IF.
LAA593Hu71 Biotin-Linked Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC.
PAA593Hu03 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; ICC; IHC-P; IHC-F; ELISA.
PAA593Hu04 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; ICC; IHC-P; IHC-F; ELISA.
PAA593Hu05 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC; IP.
PAA593Hu06 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC; IP.
Assay Kits SEA593Hu ELISA Kit for Fibrillin 1 (FBN1) Enzyme-linked immunosorbent assay for Antigen Detection.
SCA593Hu CLIA Kit for Fibrillin 1 (FBN1) Chemiluminescent immunoassay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA593Mu02 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
RPA593Mu03 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
RPA593Mu01 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
CPA593Mu21 OVA Conjugated Fibrillin 1 (FBN1) Immunogen; SDS-PAGE; WB.
APA593Mu01 Active Fibrillin 1 (FBN1) Cell culture; Activity Assays.
Antibodies PAA593Mu03 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; ICC; IHC-P; IHC-F; ELISA.
PAA593Mu02 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC; IP.
PAA593Mu01 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC; IP.
LAA593Mu71 Biotin-Linked Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC.
PAA593Mu08 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC; IP.
Assay Kits SEA593Mu ELISA Kit for Fibrillin 1 (FBN1) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA593Ra01 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
RPA593Ra02 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
RPA593Ra03 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
RPA593Ra04 Recombinant Fibrillin 1 (FBN1) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA593Ra01 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC; IP.
PAA593Ra02 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC; IP.
LAA593Ra71 Biotin-Linked Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC.
PAA593Ra03 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC; IP.
PAA593Ra04 Polyclonal Antibody to Fibrillin 1 (FBN1) WB; IHC; ICC; IP.
Assay Kits SEA593Ra ELISA Kit for Fibrillin 1 (FBN1) Enzyme-linked immunosorbent assay for Antigen Detection.
SCA593Ra CLIA Kit for Fibrillin 1 (FBN1) Chemiluminescent immunoassay for Antigen Detection.

Organism species: Bos taurus; Bovine (Cattle)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Fibrillin 1 (FBN1) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Fibrillin 1 (FBN1) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Fibrillin 1 (FBN1) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Fibrillin 1 (FBN1) CLIA Kit Customized Service Offer
n/a ELISA Kit for Fibrillin 1 (FBN1) ELISA Kit Customized Service Offer
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  28. "Marfan phenotype variability in a family segregating a missense mutation in the epidermal growth factor-like motif of the fibrillin gene."J. Clin. Invest. 89:1674-1680(1992) [PubMed] [Europe PMC] [Abstract]
  29. "Four novel FBN1 mutations: significance for mutant transcript level and EGF-like domain calcium binding in the pathogenesis of Marfan syndrome."Genomics 17:468-475(1993) [PubMed] [Europe PMC] [Abstract]
  30. "A novel fibrillin mutation in the Marfan syndrome which could disrupt calcium binding of the epidermal growth factor-like module."Hum. Mol. Genet. 2:475-477(1993) [PubMed] [Europe PMC] [Abstract]
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  39. "A Gly1127Ser mutation in an EGF-like domain of the fibrillin-1 gene is a risk factor for ascending aortic aneurysm and dissection."Am. J. Hum. Genet. 56:1287-1296(1995) [PubMed] [Europe PMC] [Abstract]
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  63. "Central nervous system abnormalities in two cases with neonatal Marfan syndrome with novel mutations in the fibrillin-1 gene."Am. J. Med. Genet. A 152:2409-2412(2010) [PubMed] [Europe PMC] [Abstract]
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