Fbn2 - fibrillin 2

Predicted to be an extracellular matrix structural constituent. Involved in several processes, including bone trabecula formation; positive regulation of osteoblast differentiation; and sequestering of TGFbeta in extracellular matrix. Acts upstream of or within embryonic limb morphogenesis. Located in extracellular region and microfibril. Is expressed in several structures, including connective tissue; embryo mesenchyme; genitourinary system; heart; and respiratory system. Used to study distal arthrogryposis. Human ortholog(s) of this gene implicated in congenital contractural arachnodactyly and scoliosis. Orthologous to human FBN2 (fibrillin 2). [provided by Alliance of Genome Resources, Apr 2022]