Anti-Fibrillin monoclonal antibody (DMAB8368)

Mouse Anti-Bovine Fibrillin monoclonal antibody for IHC, WB Datasheet

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Specifications


Host Species
Mouse
Antibody Isotype
IgG1
Clone
12D2.4
Species Reactivity
Bovine
Immunogen
Microfibrils from zonular apparatus of bovine eye
Conjugate
Unconjugated

Target


Alternative Names
FBN; SGS; WMS; MASS; MFS1; OCTD
Entrez Gene ID
UniProt ID

Product Background


Gene summary
FBN1 (Fibrillin 1) is a Protein Coding gene. Diseases associated with FBN1 include stiff skin syndrome and marfan syndrome. Among its related pathways are Degradation of the extracellular matrix and ERK Signaling. GO annotations related to this gene include calcium ion binding and extracellular matrix structural constituent. An important paralog of this gene is LTBP3. This gene encodes a member of the fibrillin family of proteins. The encoded preproprotein is proteolytically processed to generate two proteins including the extracellular matrix component fibrillin-1 and the protein hormone asprosin. 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 nonelastic connective tissue throughout the body. Asprosin, secreted by white adipose tissue, has been shown to regulate glucose homeostasis. Mutations in this gene are associated with Marfan syndrome and the related MASS phenotype, as well as ectopia lentis syndrome, Weill-Marchesani syndrome, Shprintzen-Goldberg syndrome and neonatal progeroid syndrome.

Citations


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References


McNearney, TA; Reveille, JD; et al. Pulmonary involvement in systemic sclerosis: Associations with genetic, serologic, sociodemographic, and behavioral factors. ARTHRITIS & RHEUMATISM-ARTHRITIS CARE & RESEARCH 57:318-326(2007).
Matt, P; Habashi, J; et al. Recent advances in understanding Marfan syndrome: Should we now treat surgical patients with losartan?. JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY 135:389-394(2008).

Custom Antibody Labeling


We offer labeled antibodies using our catalogue antibody products and a broad range of intensely fluorescent dyes and labels including HRP, biotin, ALP, Alexa Fluor® dyes, DyLight® Fluor dyes, R-phycoerythrin (R-PE), at scales from less than 100 μg up to 1 g of IgG antibody. Learn More

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