All mammals have three fibrillin genes (Davis & Summers 2012). Fibrillin-3 arose as a duplication of fibrillin-2 that did not occur in the rodent lineage. Fibrillin-1 is the major structural component of microfibrils (Kielty et al. 2005). Fibrillin-2 is expressed earlier in development than fibrillin-1 and may be important for elastic fiber formation (Zhang et al. 1994). Fibrillin-3 was isolated from and is predominantly expressed in brain; it is not known whether it forms microfibrils (Corson et al. 2004). Fibrillin-1 and -2 are degraded by MMP2, 9, 12 and 13 (Ashworth et al. 1999, Hindson et al. 1999). Fibrillin-1 is additionally degraded by MMP3 (Ashworth et al. 1999), the membrane-associated MMP14 (Ashworth et al. 1999), neutrophil elastase (ELANE) (Kielty et al. 1994), cathepsin L2 (V) and cathepsin K (Kirschner et al. 2011).
Shuttleworth, CA, Hindson, VJ, Cunliffe, S, Kielty, CM, Ashworth, JL, Rock, MJ
Shuttleworth, CA, Murphy, G, Sherratt, MJ, Kielty, CM, Ashworth, JL, Rock, MJ, Shapiro, SD
metalloendopeptidase activity of MMP2,9,12,13 [extracellular region]
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