| 背景 | This is clone 5G11, a mouse IgG monoclonal antibody raised against human Fibulin-4 (EFEMP2; UniProt O95967) and validated for immunofluorescence, paraffin-section immunohistochemistry and Western blot. As a bulk-format, research-use-only reagent it is well suited to matrix-biology and extracellular-matrix studies where large or repeated quantities of a single, lot-consistent antibody are needed, for example screening across tissue panels, developmental time courses, or comparisons of healthy versus fibrotic or aneurysmal specimens. The mouse host and IgG isotype make it compatible with standard anti-mouse detection systems and with co-staining alongside antibodies raised in other species. Because Fibulin-4 is a secreted, matrix-deposited glycoprotein, this clone is most informative for localising the protein within elastic-fibre-rich connective tissue and for assessing its abundance and processing by immunoblot. Supplied at low endotoxin and in bulk milligram-to-gram quantities, it supports high-throughput and multi-site experiments. It is intended for in-vitro and ex-vivo characterisation of human Fibulin-4 rather than in-vivo functional intervention.
Fibulin-4 (also known as EFEMP2, encoded by EFEMP2; UniProt O95967) is a small secreted glycoprotein of the fibulin family, characterised by tandem calcium-binding EGF-like domains and a C-terminal fibulin-type module. It is an essential organiser of elastic fibres in the extracellular matrix, acting largely as a chaperone and scaffold: it binds tropoelastin and lysyl oxidase (LOX) and promotes the crosslinking and correct deposition of elastin onto microfibrils. Fibulin-4 is abundant in elastic tissues such as the aorta, lung, skin and other connective tissues. Loss or mutation of EFEMP2 causes autosomal-recessive cutis laxa with severe vascular phenotypes, including arterial tortuosity, aneurysm and stiffness, reflecting failed elastogenesis. It also interacts with fibrillin and other matrix components and influences TGF-beta signalling in the vessel wall. Its expression and processing are of interest in cardiovascular disease, aneurysm, fibrosis and connective-tissue disorders. Fibulin-4 is predominantly expressed in tissues rich in elastic fibers. |
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