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Details on Person All mammals have three fibrillin genes (Davis & Summers 2012...

Class:IdSummation:2485097
_displayNameAll mammals have three fibrillin genes (Davis & Summers 2012...
_timestamp2012-10-10 14:43:24
created[InstanceEdit:2485082] Jupe, S, 2012-09-28
literatureReference[LiteratureReference:2514816] Structure and function of the mammalian fibrillin gene family: Implications for human connective tissue diseases
[LiteratureReference:2514797] Fibrillin microfibrils
[LiteratureReference:2514781] Structure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matrices
[LiteratureReference:2514792] Differential expression of fibrillin-3 adds to microfibril variety in human and avian, but not rodent, connective tissues
[LiteratureReference:2485133] Fibrillin degradation by matrix metalloproteinases: implications for connective tissue remodelling
[LiteratureReference:2485135] Fibrillin degradation by matrix metalloproteinases: identification of amino- and carboxy-terminal cleavage sites
[LiteratureReference:2485141] Catabolism of intact fibrillin microfibrils by neutrophil elastase, chymotrypsin and trypsin
[LiteratureReference:2514804] Classical and neonatal Marfan syndrome mutations in fibrillin-1 cause differential protease susceptibilities and protein function
modified[InstanceEdit:2514810] Jupe, S, 2012-10-10
[InstanceEdit:2514825] Jupe, S, 2012-10-10
textAll 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).
(summation)[Reaction:2485148] Fibrillin 1, 2,(3) degradation by MMP2, 9, 12 and 13 [Homo sapiens]
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