Anti-Fibrillin 1 antibody (ab53076)
Key features and details
- Rabbit polyclonal to Fibrillin 1
- Suitable for: ELISA, ICC/IF, IHC-P
- Reacts with: Mouse, Human
- Isotype: IgG
Overview
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Product name
Anti-Fibrillin 1 antibody
See all Fibrillin 1 primary antibodies -
Description
Rabbit polyclonal to Fibrillin 1 -
Host species
Rabbit -
Tested applications
Suitable for: ELISA, ICC/IF, IHC-Pmore details -
Species reactivity
Reacts with: Mouse, Human
Predicted to work with: Rat -
Immunogen
Synthetic peptide: STPLYKKKEL NQLEDKYDKD YLSGELGDNL KMKIQVLLH, corresponding to amino acids 2832-2871 of Human Fibrillin 1
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General notes
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C. -
Storage buffer
pH: 7.40
Preservative: 0.02% Sodium azide
Constituents: 50% Glycerol, 0.87% Sodium chloride, PBS
Without Mg+2 and Ca+2 -
Concentration information loading...
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Purity
Immunogen affinity purified -
Purification notes
The antibody was affinity purified from rabbit antiserum by affinity chromatography using epitope-specific immunogen. -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Recombinant Protein
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab53076 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application | Abreviews | Notes |
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ELISA |
Use at an assay dependent concentration.
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ICC/IF |
Use at an assay dependent concentration. PubMed: 23401661
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IHC-P | (1) |
Use at an assay dependent concentration.
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Notes |
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ELISA
Use at an assay dependent concentration. |
ICC/IF
Use at an assay dependent concentration. PubMed: 23401661 |
IHC-P
Use at an assay dependent concentration. |
Target
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Function
Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-1-containing microfibrils provide long-term force bearing structural support. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. -
Involvement in disease
Defects in FBN1 are a cause of Marfan syndrome (MFS) [MIM:154700]. MFS is an autosomal dominant disorder that affects the skeletal, ocular, and cardiovascular systems. A wide variety of skeletal abnormalities occurs with MFS, including scoliosis, chest wall deformity, tall stature, abnormal joint mobility. Ectopia lentis occurs in up to about 80% of MFS patients and is almost always bilateral. The leading cause of premature death in MFS patients is progressive dilation of the aortic root and ascending aorta, causing aortic incompetence and dissection. Note=The majority of the more than 600 mutations in FBN1 currently known are point mutations, the rest are frameshifts and splice site mutations. Marfan syndrome has been suggested in at least 2 historical figures, Abraham Lincoln and Paganini.
Defects in FBN1 are a cause of isolated ectopia lentis (EL) [MIM:129600]. The symptoms of this autosomal dominant fibrillinopathy overlap with those of Marfan syndrome, with the exclusion of the skeletal and cardiovascular manifestations.
Defects in FBN1 are the cause of Weill-Marchesani syndrome autosomal dominant (ADWMS) [MIM:608328]. A rare connective tissue disorder characterized by short stature, brachydactyly, joint stiffness, and eye abnormalities including microspherophakia, ectopia lentis, severe myopia and glaucoma.
Defects in FBN1 are a cause of Shprintzen-Goldberg craniosynostosis syndrome (SGS) [MIM:182212]. SGS is a very rare syndrome characterized by a marfanoid habitus, craniosynostosis, characteristic dysmorphic facial features, skeletal and cardiovascular abnormalities, mental retardation, developmental delay and learning disabilities.
Defects in FBN1 are a cause of overlap connective tissue disease (OCTD) [MIM:604308]. A heritable disorder of connective tissue characterized by involvement of the mitral valve, aorta, skeleton, and skin. MASS syndrome is closely resembling both the Marfan syndrome and the Barlow syndrome. However, no dislocation of the lenses or aneurysmal changes occur in the aorta, and the mitral valve prolapse is by no means invariable.
Defects in FBN1 are a cause of stiff skin syndrome (SSKS) [MIM:184900]. It is a syndrome characterized by hard, thick skin, usually over the entire body, which limits joint mobility and causes flexion contractures. Other occasional findings include lipodystrophy and muscle weakness. -
Sequence similarities
Belongs to the fibrillin family.
Contains 47 EGF-like domains.
Contains 9 TB (TGF-beta binding) domains. -
Post-translational
modificationsForms intermolecular disulfide bonds either with other fibrillin-1 molecules or with other components of the microfibrils. -
Cellular localization
Secreted > extracellular space > extracellular matrix. - Information by UniProt
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Database links
- Entrez Gene: 2200 Human
- Entrez Gene: 83727 Rat
- Omim: 134797 Human
- SwissProt: P35555 Human
- SwissProt: Q61554 Mouse
- Unigene: 591133 Human
- Unigene: 271644 Mouse
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Alternative names
- 350 kDa glycoprotein component extracellular microfibril antibody
- ACMICD antibody
- FBN 1 antibody
see all
Images
Protocols
Datasheets and documents
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SDS download
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Datasheet download
References (20)
ab53076 has been referenced in 20 publications.
- Ortega MA et al. Contribution of the Elastic Component and Venous Wall Arterialization in Patients with Venous Reflux. J Pers Med 12:N/A (2022). PubMed: 35207748
- Federspiel JM et al. Two Cases of Quadricuspid Aortic Valve: Aortic Regurgitation and Degeneration. Thorac Cardiovasc Surg Rep 11:e39-e43 (2022). PubMed: 35865148
- Balint B et al. SMAD3 contributes to ascending aortic dilatation independent of transforming growth factor-beta in bicuspid and unicuspid aortic valve disease. Sci Rep 12:15476 (2022). PubMed: 36104385
- Boëté Q et al. Physiological Impact of a Synthetic Elastic Protein in Arterial Diseases Related to Alterations of Elastic Fibers: Effect on the Aorta of Elastin-Haploinsufficient Male and Female Mice. Int J Mol Sci 23:N/A (2022). PubMed: 36362244
- Yu X et al. Nanophthalmos-Associated MYRF Gene Mutation Causes Ciliary Zonule Defects in Mice. Invest Ophthalmol Vis Sci 62:1 (2021). PubMed: 33646289