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Home >> Antibodies >> Fibrillin-1 (FBN1) (Marfan Syndrome Marker)

Fibrillin-1 (FBN1) (Marfan Syndrome Marker)

Mouse Monoclonal Antibody [Clone FBN1/6933]

Catalog #

Size

20 ug
100 ug
100 ug

Price (USD)

219.00
499.00
499.00

Formulation

Purified Ab with BSA and Azide at 200ug/ml
Purified Ab with BSA and Azide at 200ug/ml
Purified Ab WITHOUT BSA and Azide at 1.0mg/ml

Catalog No

Size

20 ug
100 ug
100 ug

Formulation

Purified Ab with BSA and Azide at 200ug/ml
Purified Ab with BSA and Azide at 200ug/ml
Purified Ab WITHOUT BSA and Azide at 1.0mg/ml

Price (USD)

219.00
499.00
499.00
img

Formalin-fixed, paraffin-embedded human kidney carcinoma stained with Fibrillin 1 Mouse Monoclonal Antibody (FBN1/6933). HIER: Tris/EDTA, pH9.0, 45min. 2: HRP-polymer, 30min. DAB, 5min.

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Formalin-fixed, paraffin-embedded human prostate stained with Fibrillin 1 Mouse Monoclonal Antibody (FBN1/6933). HIER: Tris/EDTA, pH9.0, 45min. 2: HRP-polymer, 30min. DAB, 5min.

img

Formalin-fixed, paraffin-embedded human colon carcinoma stained with Fibrillin 1 Mouse Monoclonal Antibody (FBN1/6933). HIER: Tris/EDTA, pH9.0, 45min. 2: HRP-polymer, 30min. DAB, 5min.

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SDS-PAGE Analysis Purified Fibrillin 1 Mouse Monoclonal Antibody (FBN1/6933). Confirmation of Purity and Integrity of Antibody.

Product Details

Synonyms

350kDa glycoprotein component extracellular microfibril; ACMICD; FBN1; Fibrillin-15; Fibrillin-1; GPHYSD2; Marfan syndrome; MASS; MFS1; OCTD; SGS; SSKS; Weill Marchesani syndrome; WMS; WMS2

Positive Control

HepG2 cells. Human kidney or placenta.

Known Applications & Suggested Dilutions

ELISA, IHC-P         More Details

ELISA (For coating, order antibody without BSA)
Immunohistochemistry (Formalin-fixed) (1-2ug/ml for 30 minutes at RT)
(Staining of formalin-fixed tissues requires heating tissue sections in 10mM Tris with 1mM EDTA, pH 9.0, for 45 min at 95 °C followed by cooling at RT for 20 minutes)
Optimal dilution for a specific application should be determined.

Immunogen

Recombinant human Fibrillin 1 fragment around aa 330-476 (Exact sequence is proprietary)

Cellular Localization

Extracellular matrix.

Species Reactivity

Human.

Host / Ig Isotype

Mouse / IgG1, kappa

Mol. Weight of Antigen

350kDa

Specificity & Comments

Fibrillin-1 is an extracellular matrix glycoprotein that serves as a structural component of calcium-binding microfibrils. These microfibrils provide force-bearing structural support in elastic and non-elastic connective tissue throughout the body.Mutations in this gene are associated with Marfan syndrome.

Related Products



200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/ml.

Key References

  1. Mariencheck, M.C., et al. 1995. Fibrillin-1 and fibrillin-2 show temporal and tissue-specific regulation of expression in developing elastic tissues. Connect. Tissue Res. 31: 87-97.

Bioinformatics

  •  Entrez Gene ID: 2200
  •  Gene Symbol: FBN1
  •  SwissProt: P35555
  •  Chromosome Location: 15q21.1
  •  Unigene: 591133