Barbara Gayrd is an amic rearcher om Hospal for Special Surgery. The thor has ntributed to rearch topic(s): Loeys–Dietz syndrome & Marfan syndrome. The thor has an hx of 1, -thored 1 publitn(s) receivg 1188 catn(s).
Contents:
BARBARA GAYRD-MOREL
Gayrd-Morel B, Le Bouteiller M, Commere PH, Cohen-Tannoudji M, Tajbakhsh S,. Formila L, Pannérec A, Correra RM, Gayrd-Morel B, Ollrlt D, Bson V, Tajbakhsh S, Lachey J, Seehra JS, Marazzi G, Sassoon DA,. Here, the term “antimorph” will be ed to scribe the domant negative activy of stcturally fective fibrill 1 molecul on the wild-type l of mutant fibrill 1 mice that were created by homologo gene targetg have recently refed and extend this pathogenic mol (Pereira et al.
On the other hand, homozygo mgΔ animals die of MFS-like vascular plitns wh the first month of postnatal life as a rult of substantial fibrill 1 ficiency. Homozygo Tsk embryos generate utero at ∼8 d of gtatn of unknown (Green et al. Our rults exclu the assembly of distct homotypic fibrill 1 polymers Tsk/+ mice and, stead, suggt the followg mol (see Fig.
Immunoprecipatn and Fibrill 1 ProteolysisFor immunoprecipatn, subnfluent cells were grown for 24 h Met/Cys-ee DME supplemented wh 50 μCi/ml of [35S]Cys/Met mixture (ICN Bmedils; Gayrd et al. Trichrome-staed sk sectns om adult (a and b) and newborn (c and d) mice heterozygo for the Tsk allele (a), homozygo for the mgR allele (b), pound heterozygo for the Tsk and mgR allel (c), and om wild-type (+/+) animals (d). (b) Wtern blot analysis unr native ndns of medium of cell cultur om wild-type (+/+) and heterozygo (Tsk/+) and homozygo (Tsk/Tsk) mutant animals wh antibodi agast the NH2 term of fibrill 1 (pAb9543 or α-Fbn) and the mutant Tsk fibrill 1 (pAb8368 or α-Tsk).
BARBARA GAYRD
DiscsnOur current unrstandg of the role of fibrill 1 microfibrils nnective tissue physlogy is based primarily on the study of human mutatns MFS and homologo gene targetg the moe (Dietz and Pyerz 1995; Ramirez et al. Therefore, the cultured system should be viewed more as a bchemil bdg assay to tt homopolymerizatn vers heteropolymerizatn rather than a physlogil mol of microfibril assembly.
The phenotypic severy of Tsk and mgΔ homozygot is dramatilly different.