Biotin Anti-Fibrillin 1 antibody [11C1.3] (ab24826)
Key features and details
- Biotin Mouse monoclonal [11C1.3] to Fibrillin 1
- Suitable for: IHC-Fr, ICC
- Reacts with: Mouse, Human
- Conjugation: Biotin
- Isotype: IgG1
Overview
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Product name
Biotin Anti-Fibrillin 1 antibody [11C1.3]
See all Fibrillin 1 primary antibodies -
Description
Biotin Mouse monoclonal [11C1.3] to Fibrillin 1 -
Host species
Mouse -
Conjugation
Biotin -
Specificity
This antibody recognises fibrillin (Mr 350 kDa). -
Tested applications
Suitable for: IHC-Fr, ICCmore details -
Species reactivity
Reacts with: Mouse, Human -
Immunogen
Tissue, cells or virus corresponding to Cow Fibrillin 1. Microfibrils from the zonular apparatus of bovine eye
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Positive control
- IHC-Fr: Mouse skin tissue section; ICC: MIA PaCa-2 cells.
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. Store In the Dark. -
Storage buffer
pH: 7.4
Preservative: 0.1% Sodium azide
Constituent: PBS -
Concentration information loading...
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Purity
Affinity purified -
Clonality
Monoclonal -
Clone number
11C1.3 -
Isotype
IgG1 -
Light chain type
kappa -
Research areas
Associated products
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Isotype control
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Recombinant Protein
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab24826 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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IHC-Fr |
Use at an assay dependent concentration.
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ICC |
Use at an assay dependent concentration.
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Notes |
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IHC-Fr
Use at an assay dependent concentration. |
ICC
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
- 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
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Immunocytochemistry analysis of MIA PaCa-2 (human pancreatic carcinoma cell line) cells labeling Fibrillin 1 with ab24826 (right) followed by Streptavidin-CY3.5 (red). Biotinylated Mouse IgG1 used as the isotype control (left) followed by Streptavidin-CY3.5 (red). Nuclei counterstained with DAPI (blue).
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Immunohistochemistry analysis of frozen mouse skin tissue sections labeling Fibrillin 1 with ab24826 (right) followed by Streptavidin-CY3.5 (red). Biotinylated Mouse IgG1 used as the isotype control (left). Nuclei counterstained with DAPI (blue).
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
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SDS download
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Datasheet download
References (3)
ab24826 has been referenced in 3 publications.
- Vidali S et al. Thyroid Hormones Enhance Mitochondrial Function in Human Epidermis. J Invest Dermatol 136:2003-2012 (2016). PubMed: 27349864
- Maier A et al. Fibrillin and elastin networks in extrafusal tissue and muscle spindles of bovine extraocular muscles. Invest Ophthalmol Vis Sci 35:3103-10 (1994). PubMed: 8206729
- Wright DW et al. Immunization with undenatured bovine zonular fibrils results in monoclonal antibodies to fibrillin. Matrix Biol 14:41-9 (1994). PubMed: 8061919