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Fibrillin

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Fibrillin-1: Structural component of the 10-12 nm diameter microfibrils of the extracellular matrix, which conveys both structural and regulatory properties to load-bearing connective tissues .

Below are the list of possible Fibrillin products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

Fibrillin-1

 Fibrillin-1 ELISA Kit
 Fibrillin-1 Recombinant
 Fibrillin-1 Antibody
FBN1: a structural component of the 10-12 nm diameter microfibrils of the extracellular matrix, which conveys both structural and regulatory properties to load-bearing connective tissues. FBN1-containing microfibrils provide long-term force bearing structural support. In tissues such as the lung, blood vessels and skin, microfibrils form the periphery of the elastic fiber, acting as a scaffold for
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the deposition of elastin. In addition, microfibrils can occur as elastin-independent networks in tissues such as the ciliary zonule, tendon, cornea and glomerulus where they provide tensile strength and have anchoring roles. Plays a key role in tissue homeostasis through specific interactions with growth factors, such as the bone morphogenetic proteins (BMPs), growth and differentiation factors and latent transforming growth factor-beta-binding proteins, cell-surface integrins and other extracellular matrix protein and proteoglycan components. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. Negatively regulates osteoclastogenesis by binding and sequestering an osteoclast differentiation and activation factor TNFSF11. This leads to disruption of TNFSF11-induced Ca2+ signaling and impairment of TNFSF11-mediated nuclear translocation and activation of transcription factor NFATC1 which regulates genes important for osteoclast differentiation and function. Mediates cell adhesion via its binding to cell surface receptors integrins ITGB3 and ITGB1. Binds heparin and this interaction has an important role in the assembly of microfibrils. Cleavage of N- and C-termini is required for incorporation into the extracellular matrix and assembly into microfibrils. Its cleaved C-terminal peptide (2732 ¿ 2871) is the hormone asprosin, secreted by white adipose tissue that targets the liver, stimulating the release of glucose and increasing plasma glucose levels. Acts in response to fasting, and promotes hepathocyte glucose release via a PKA-dependent pathway.

Protein type: Extracellular matrix; Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 15q21.1

Cellular Component: basement membrane; extracellular matrix; extracellular region; extracellular space; microfibril; proteinaceous extracellular matrix

Molecular Function: calcium ion binding; extracellular matrix constituent conferring elasticity; extracellular matrix structural constituent; heparin binding; integrin binding; protein binding; protein complex binding

Biological Process: camera-type eye development; cell adhesion mediated by integrin; embryonic eye morphogenesis; extracellular matrix disassembly; extracellular matrix organization and biogenesis; heart development; negative regulation of osteoclast differentiation; post-embryonic eye morphogenesis; skeletal development

Disease: Acromicric Dysplasia; Ectopia Lentis 1, Isolated, Autosomal Dominant; Geleophysic Dysplasia 2; Marfan Lipodystrophy Syndrome; Marfan Syndrome; Mass Syndrome; Stiff Skin Syndrome; Weill-marchesani Syndrome 2
 FBN1 ELISA Kit
 FBN1 Recombinant
 FBN1 Antibody
 FBN ELISA Kit
 FBN Recombinant
 FBN Antibody
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Fibrillin-2

 Fibrillin-2 ELISA Kit
 Fibrillin-2 Recombinant
 Fibrillin-2 Antibody
FBN2: 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-2- containing microfibrils regulate the early process of elastic fiber assembly. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. Defects
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in FBN2 are the cause of distal arthrogryposis type 9 (DA9). A form of distal arthrogryposis, a disease characterized by congenital joint contractures that mainly involve two or more distal parts of the limbs, in the absence of a primary neurological or muscle disease. DA9 is a connective tissue disorder characterized by contractures, arachnodactyly, scoliosis, and crumpled ears. Belongs to the fibrillin family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 5q23-q31

Cellular Component: extracellular matrix; extracellular region; microfibril

Molecular Function: extracellular matrix constituent conferring elasticity; extracellular matrix structural constituent; protein binding

Biological Process: camera-type eye development; embryonic eye morphogenesis; extracellular matrix disassembly; extracellular matrix organization and biogenesis; positive regulation of bone mineralization; positive regulation of osteoblast differentiation

Disease: Arthrogryposis, Distal, Type 9; Macular Degeneration, Early-onset
 FBN2 ELISA Kit
 FBN2 Recombinant
 FBN2 Antibody
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Fibrillin-3

 Fibrillin-3 ELISA Kit
 Fibrillin-3 Recombinant
 Fibrillin-3 Antibody
FBN3: 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- containing microfibrils provide long-term force bearing structural support. Belongs to the fibrillin family.

Protein type: Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 19p
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Cellular Component: extracellular matrix

Molecular Function: extracellular matrix structural constituent

Biological Process: anatomical structure morphogenesis
 FBN3 ELISA Kit
 FBN3 Recombinant
 FBN3 Antibody
 KIAA1776 ELISA Kit
 KIAA1776 Recombinant
 KIAA1776 Antibody
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