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Fibrillin 1 Proteins (FBN1)

FBN1 encodes a member of the fibrillin family. Additionally we are shipping Fibrillin 1 Antibodies (149) and Fibrillin 1 Kits (38) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
FBN1 2200 P35555
FBN1 14118  
FBN1 83727  
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Top Fibrillin 1 Proteins at antibodies-online.com

Showing 7 out of 20 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
HOST_Wheat germ Human GST tag 10 μg Log in to see 9 Days
$405.71
Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Mouse His tag 100 μg Log in to see 11 to 13 Days
$823.90
Details
HOST_Escherichia coli (E. coli) Human His tag 100 μg Log in to see 11 to 13 Days
$855.80
Details
HOST_Escherichia coli (E. coli) Rat His tag 100 μg Log in to see 11 to 13 Days
$862.40
Details
HOST_Escherichia coli (E. coli) Rat Un-conjugated   100 μg Log in to see 9 to 16 Days
$900.63
Details
HOST_Escherichia coli (E. coli) Human Un-conjugated   100 μg Log in to see 9 to 16 Days
$900.63
Details

FBN1 Proteins by Origin and Source

Origin Expressed in Conjugate
Human ,
, ,
Mouse (Murine)

Rat (Rattus)

Top referenced Fibrillin 1 Proteins

  1. Human Fibrillin 1 Protein expressed in Wheat germ - ABIN1353704 : Atanasova, Konova, Aleksovska, Todorova, Georgieva, Lukanov: Anti-fibrillin-1 autoantibodies in normal pregnancy and recurrent pregnancy loss. in Autoimmunity reviews 2011 (PubMed)

More Proteins for Fibrillin 1 (FBN1) Interaction Partners

Cow (Bovine) Fibrillin 1 (FBN1) interaction partners

  1. Data suggest that genetic fibrillin-1 deficiency could alter normal endothelial signaling.

  2. In cases of vascular calcification, the decreased expression of FBN1 may be partially responsible for decreased vascular elasticity and also for the decreased formation of new elastic fibers.

  3. The coordinate upregulation of fibrillin-1 and fibrillin-2 (show FBN2 Proteins) expression with the onset of tropoelastin (show ELN Proteins) production is consistent with a role in elastic fiber assembly.

  4. a calcium-binding epidermal growth factor (show EGF Proteins)-like domain of fibrillin-1 c.3598G > A, p.E1200K mutation is responsible for a bovine model of Marfan syndrome

  5. This is the first study to investigate the expression and localization of fibrillin proteins and latent TGF-beta (show TGFB1 Proteins) binding proteins affecting TGFbeta (show TGFB1 Proteins) bioavailability in the ovary.

Human Fibrillin 1 (FBN1) interaction partners

  1. This report broadens the phenotypic spectrum of growth disorders associated with FBN1 mutations. Identical mutations give rise to a wide phenotypic spectrum, ranging from isolated short stature to a more classic picture of GD2 with cardiac involvement, distinct facial dysmorphisms and various skeletal anomalies.

  2. A subgroup of patients with Marfan syndrome (MFS) who have mutations in exons 24-32 of the FBN1 gene manifests severe atrioventricular valve insufficiency and skeletal problems as early as the neonatal period. These patients usually die in the first 2 years of life, thus a region between exons 24 and 32 of FBN1 is recognized as a critical region for this neonatal form of MFS (nMFS). [review, case reports]

  3. FBN1 gene mutation is associated with Marfan syndrome.

  4. Results showed two novel mutations in exon 12 and 50 of FBN1 identified in two Chinese family members with Marfan syndrome (MFS) which may be responsible for cardiovascular manifestations.

  5. The results expand the FBN1 mutation spectrum and enrich our knowledge of genotype-phenotype correlations. Genetic testing for MFS and its related aortic diseases is increasingly important for early intervention and treatment.

  6. miR (show MLXIP Proteins)-486-5p induces papillary thyroid carcinoma cell growth inhibition and apoptosis by targeting and suppressing FBN1.

  7. information of genotype-phenotype correlation owing to FBN1 mutations; the same FBN1 mutation, c. 1633C>T (Arg545Cys), was detected simultaneously in three different cardinal (show CARD8 Proteins) phenotypes (ectopia lentis, aortic dissection and unaffected) within one family.

  8. Data show that interaction of fibrillin-1 with the bone morphogenetic protein 7 (BMP-7 (show BMP7 Proteins)) complex results in a conformational change.

  9. Authors discovered a protein hormone that regulates glucose homeostasis. It is the C-terminal cleavage product of profibrillin (encoded by FBN1). Its absence in humans results in a unique pattern of metabolic dysregulation that includes partial lipodystrophy, accompanied by reduced plasma insulin (show INS Proteins), while maintaining euglycemia.

  10. A marked decrease in heart rate variability, documented in the study, may be an important clinical feature in MS patients with confirmed FBN1 mutations.

Mouse (Murine) Fibrillin 1 (FBN1) interaction partners

  1. These results suggest that both fibrillin-1 and fibrillin-2 (show FBN2 Proteins) expression is required to form thick oxytalan fibers in periodontal ligament.

  2. findings show that fibrillin-1 regulates MSC (show MSC Proteins) activity by modulating TGFbeta (show TGFB1 Proteins) bioavailability within the microenvironment of marrow niches.

  3. Fibrillin-1 mgDelta(lpn) Marfan syndrome mutation associates with preserved proteostasis and bypass of a protein disulfide isomerase (show P4HB Proteins)-dependent quality checkpoint

  4. fibrillin-1 deficiency is associated with relevant dysfunction of the endothelial barrier that enables adenovirus to induce vessel-harming inflammation.

  5. the Fbn1(C1039G/+) mouse model demonstrates mild intrinsic left ventricular dysfunction

  6. This study demonstrated that dysfunctional fibrillin-1 impairs blood-brain barrier permeability /BCSFB integrity, facilitating peripheral leukocyte infiltration, which further degrades the BBB/BCSFB.

  7. The review discuss FBN1 point mutations on the structure and function of the protein associated with Marfan syndrome in affected patients impairing protein folding and traficking, secretion, proteolysis or heparin binding.

  8. The concomitant induction of both fibrillin-1 and alpha8 integrin in a self-limited model of glomerular injury points to a protective role of the interaction of fibrillin-1 with alpha8 integrin in the glomerulus.

  9. Data show that fibrillin-1 (FBN1) modulates bone marrow mesenchymal stem cells (BMMSCs) lineage differentiation via IL4 receptor alpha (show IL4R Proteins)/mTOR (show FRAP1 Proteins) protein signaling.

  10. latent transforming growth factor-beta-1 (show TGFB1 Proteins) binding protein-2 was prominently associated with annular fibrils containing fibrillin-1

Fibrillin 1 (FBN1) Protein Profile

Protein Summary

This gene encodes a member of the fibrillin family. The encoded protein is a large, extracellular matrix glycoprotein that serve as a structural component of 10-12 nm calcium-binding microfibrils. These microfibrils provide force bearing structural support in elastic and nonelastic connective tissue throughout the body. Mutations in this gene are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome.

Gene names and symbols associated with Fibrillin 1 Proteins (FBN1)

  • fibrillin 1 (FBN1)
  • fibrillin 1 (Fbn1)
  • fibrillin 1-like (LOC100350931)
  • ACMICD protein
  • AI536462 protein
  • B430209H23 protein
  • ECTOL1 protein
  • FBN protein
  • FBN1 protein
  • Fib-1 protein
  • GPHYSD2 protein
  • MASS protein
  • MFS1 protein
  • OCTD protein
  • SGS protein
  • SSKS protein
  • Tsk protein
  • WMS protein
  • WMS2 protein

Protein level used designations for Fibrillin 1 Proteins (FBN1)

fibrillin 1 , MP340 , fibrillin-1 , fibrillin 15 , fibrillin 1 (Marfan syndrome) , tight skin

GENE ID SPECIES
453411 Pan troglodytes
100055741 Equus caballus
281154 Bos taurus
2200 Homo sapiens
478293 Canis lupus familiaris
414836 Sus scrofa
14118 Mus musculus
83727 Rattus norvegicus
100717565 Cavia porcellus
373992 Gallus gallus
101109620 Ovis aries
100350931 Oryctolagus cuniculus
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