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Fibrillin 2

fibrillin-2, FBN2
The protein encoded by this gene is a component of connective tissue microfibrils and may be involved in elastic fiber assembly. Mutations in this gene cause congenital contractural arachnodactyly. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: fibrillin-1, elastin, CAN, HAD, fibronectin
Papers on fibrillin-2
Forelimb contractures and abnormal tendon collagen fibrillogenesis in fibulin-4 null mice.
New
Chu et al., Philadelphia, United States. In Cell Tissue Res, Jan 2016
Fibulin-4 was co-localized with fibrillin-1 and fibrillin-2 in limb tendons by using immunofluorescence microscopy.
Decreased elastic fibers and increased proteoglycans in the ligamentum flavum of patients with lumbar spinal canal stenosis.
New
Itoi et al., Sendai, Japan. In J Orthop Res, Jan 2016
The gene and protein expressions of fibrillin-2 and DANCE were significantly lower and decorin, lumican, osteoglycin, and versican were significantly higher in the thickened flavum group.
Marked disruption and aberrant regulation of elastic fibres in early striae gravidarum.
New
Fisher et al., Ann Arbor, United States. In Br J Dermatol, Dec 2015
The emergence of these fibrils was accompanied by increased gene expression of tropoelastin and fibrillin-1, but not other elastic fibre components, including fibrillin-2 and fibulin-1, -2 or -5.
Esophageal Cancer Epigenomics and Integrome Analysis of Genome-Wide Methylation and Expression in High Risk Northeast Indian Population.
New
Saxena et al., New Delhi, India. In Omics, Nov 2015
These included four genes (PTK2, RND1, RND3, and UBL3) with promoter hypermethylation and downregulation, and 19 genes (SEMG2, CD97, CTNND2, CADM3, OMD, NEFM, FBN2, CTNNB1, DLX6, UGT2B4, CCDC80, PZP, SERPINA4, TNFSF13B, NPC1, COL1A1, TAC3, BMP8A, and IL22RA2) with promoter hypomethylation and upregulation.
Analysis of the differential secretome of nasopharyngeal carcinoma cell lines CNE-2R and CNE-2.
New
Zhu et al., Nanning, China. In Oncol Rep, Nov 2015
To verify the reliability of iTRAQ quantitative proteomics, we applied western blotting (WB) to study the secretory protein expression of fibrillin-2, CD166, sulfhydryl oxidase 1 and cofilin-2, which are involved in cell adhesion, migration and invasion.
Routine Genetic Testing for Thoracic Aortic Aneurysm and Dissection in a Clinical Setting.
New
Elefteriades et al., New Haven, United States. In Ann Thorac Surg, Nov 2015
The following 21-gene panel was tested by WES: ACTA2, ADAMTS10, COL1A1, COL1A2, COL3A1, COL5A1, COL5A2, ELN, FBLN4, FLNA, FBN1, FBN2, MYH11, MYLK, NOTCH1, PRKG1, SLC2A10, SMAD3, TGFB2, TGFBR1, TGFBR2.
Fibrillin microfibrils in bone physiology.
Review
New
Ramirez et al., New York City, United States. In Matrix Biol, Oct 2015
UNASSIGNED: The severe skeletal abnormalities associated with Marfan syndrome (MFS) and congenital contractural arachnodactyly (CCA) underscore the notion that fibrillin assemblies (microfibrils and elastic fibers) play a critical role in bone formation and function in spite of representing a low abundance component of skeletal matrices.
Abnormal Activation of BMP Signaling Causes Myopathy in Fbn2 Null Mice.
New
Sakai et al., New York City, United States. In Plos Genet, Jun 2015
These new findings demonstrate that loss of fibrillin-2 results in phenotypes similar to those found in congenital muscular dystrophies and that FBN2 should be considered as a candidate gene for recessive congenital muscular dystrophy.
The Role of Immunohistochemistry in Rhabdomyosarcoma Diagnosis Using Tissue Microarray Technology and a Xenograft Model.
Llombart-Bosch et al., Valencia, Spain. In Fetal Pediatr Pathol, 2014
Desmin, myogenin, CK, EMA, INI1, LSD1, AP2β, fibrillin-2, HMGA2, nestin, and SIRT1 were tested using immunohistochemical staining.
Structure and function of the mammalian fibrillin gene family: implications for human connective tissue diseases.
Review
Summers et al., Edinburgh, United Kingdom. In Mol Genet Metab, 2012
Fibrillinopathies include Marfan syndrome, familial ectopia lentis, familial thoracic aneurysm (mutations of FBN1) and congenital contractural arachnodactyly (mutation of FBN2).
Fibrillin-1 and fibrillin-2 are essential for formation of thick oxytalan fibers in human nonpigmented ciliary epithelial cells in vitro.
GeneRIF
Ishikawa et al., Fukuoka, Japan. In Connect Tissue Res, 2011
These results suggest that both fibrillin-1 and fibrillin-2 are essential for the formation of thick oxytalan fibers in the ciliary zonule.
Material and mechanical properties of bones deficient for fibrillin-1 or fibrillin-2 microfibrils.
GeneRIF
Ramirez et al., United States. In Matrix Biol, 2011
fibrillin-2 exerts a greater positive influence on the mechanical properties of bone than fibrillin-1 assemblies
Fibrillin-1 and -2 differentially modulate endogenous TGF-β and BMP bioavailability during bone formation.
GeneRIF
Ramirez et al., New York City, United States. In J Cell Biol, 2010
Fibrillin-1 and -2, the structural components of extracellular microfibrils, differentially regulate TGF-beta and bone morphogenetic protein (BMP) bioavailability in bone.
Recent molecular biological progress in Marfan syndrome and Marfan-associated disorders.
Review
Zhou et al., Beijing, China. In Ageing Res Rev, 2010
Mutations in the fibrillin-2 gene were discovered in individuals with a phenotypically related disorder, congenital contractural arachnodactyly.
Microfibril structure masks fibrillin-2 in postnatal tissues.
GeneRIF
Sakai et al., Portland, United States. In J Biol Chem, 2010
Data demonstrated that N-terminal fibrillin-2 epitopes are masked in postnatal microfibrils.
Enhanced fibrillin-2 expression is a general feature of wound healing and sclerosis: potential alteration of cell attachment and storage of TGF-beta.
GeneRIF
Reinhardt et al., Lübeck, Germany. In Lab Invest, 2010
Data show that in wound healing and sclerotic skin diseases, a marked increase of fibrillin-2 expression was found by immunohistology.
Biogenesis and function of fibrillin assemblies.
Review
Sakai et al., New York City, United States. In Cell Tissue Res, 2010
Fibrillin-1 and fibrillin-2 are large cysteine-rich glycoproteins that serve two key physiological functions: as supporting structures that impart tissue integrity and as regulators of signaling events that instruct cell performance.
Subtype and prognostic classification of rhabdomyosarcoma by immunohistochemistry.
Impact
Schäfer et al., Zürich, Switzerland. In J Clin Oncol, 2006
RESULTS: AP2beta and P-cadherin were selected as markers for pARMS, and epidermal growth factor receptor (EGFR) and fibrillin-2 as markers for ERMS.
Congenital Contractural Arachnodactyly
Review
Godfrey, Seattle, United States. In Unknown Journal, 2001
Mutations in FBN2 (encoding the extracellular matrix microfibril fibrillin 2) are causative.
Fibrillin-2 (FBN2) mutations result in the Marfan-like disorder, congenital contractural arachnodactyly.
Impact
Milewicz et al., Houston, United States. In Nat Genet, 1995
Our study provides final proof of the association between FBN2 mutations and CCA pathology, thus establishing the role of the fibrillin-2 in extracellular matrix physiology and pathology.
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