Fibrillin 2 (FBN2)

CCA; DA9; FBLN2; Congenital Contractural Arachnodactyly; Fibrillin 5

Fibrillin 2 (FBN2)

Using the partial FBN2 cDNA isolated by Lee et al. (1991) to screen an MG-63 human osteosarcoma cell line cDNA library, Zhang et al. (1994) obtained a full-length FBN2 clone. The deduced 2,889-amino acid protein contains an N-terminal signal peptide, followed by a proline-rich region, 3 tandem N-terminal EGF-like repeats, and 41 calcium-binding EGF-like repeats interspersed with another EGF-like repeat, a glycine-rich region, 7 TGF-binding protein repeats, and 2 Fib motifs.

FBN2 also has 2 potential cell attachment sequences, multiple N-glycosylation sites, and 2 C-terminal polylysine stretches. The structures of FBN2 and FBN1 are highly similar, with the most significant difference being the glycine-rich sequence near the N terminus of FBN2, where FBN1 has a proline-rich sequence.

Organism species: Homo sapiens (Human)

Organism species: Mus musculus (Mouse)

Organism species: Rattus norvegicus (Rat)