Anti-Fibrillin-2 Antibody, clone 48

Descrição detalhada : Fibrillin-2 is a part of the Fibrillin family of glycoproteins, which are essential factors in the formation and maintenance of elastic fibers known as microfibrils within the extracellular matrix. Various researchers have suggested that Fibrillin-2 has a role in the mediation of microfibril assembly during early elastogenesis. Defects in Fibrillin-2 are causal to a rare disorder involving autosomal dominant connective tissue known as congenital contractual arachnodactyly (CCA), also known as Beals syndrome or distal arthrogryposis type 9 (DA9), and is similar to Marfan syndrome.
Sinónimos : fibrillin 2, congenital contractural arachnodactyly, Fibrillin 5
Armazenamento : +2°C to +8°C
Embalagem : 1X1EA