A microfibril assembly assay identifies different mechanisms of dominance underlying Marfan syndrome, stiff skin syndrome and acromelic dysplasias

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Marfan syndrome: current perspectives Fibrillin microfibrils in bone physiology.Fell-Muir Lecture: Fibrillin microfibrils: structural tensometers of elastic tissues?Cervical artery dissection expands the cardiovascular phenotype in FBN1-related Weill-Marchesani syndrome.

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P2860

A microfibril assembly assay identifies different mechanisms of dominance underlying Marfan syndrome, stiff skin syndrome and acromelic dysplasias

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A microfibril assembly assay i ...... drome and acromelic dysplasias

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A microfibril assembly assay i ...... drome and acromelic dysplasias

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A microfibril assembly assay i ...... drome and acromelic dysplasias

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P1476

A microfibril assembly assay i ...... drome and acromelic dysplasias

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Alicja Bulsiewicz

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Penny A Handford

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Sarah Iqbal

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P2860

Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome

Heparin/heparan sulfate controls fibrillin-1, -2 and -3 self-interactions in microfibril assembly

Latent transforming growth factor beta-binding protein 1 interacts with fibrillin and is a microfibril-associated protein

ADAMTSL2 mutations in geleophysic dysplasia demonstrate a role for ADAMTS-like proteins in TGF-beta bioavailability regulation

Structure of the integrin binding fragment from fibrillin-1 gives new insights into microfibril organization

Biophysical characterisation of fibulin-5 proteins associated with disease

In frame fibrillin-1 gene deletion in autosomal dominant Weill-Marchesani syndrome

Microenvironmental regulation by fibrillin-1

Solution structure of the third TB domain from LTBP1 provides insight into assembly of the large latent complex that sequesters latent TGF-beta

alphaVbeta6 is a novel receptor for human fibrillin-1. Comparative studies of molecular determinants underlying integrin-rgd affinity and specificity

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4454-4463

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scholarly article

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10.1093/HMG/DDV181

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Sacha Jensen

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A microfibril assembly assay identifies different mechanisms of dominance underlying Marfan syndrome, stiff skin syndrome and acromelic dysplasias

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P2860

P2860

A microfibril assembly assay identifies different mechanisms of dominance underlying Marfan syndrome, stiff skin syndrome and acromelic dysplasias

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type

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P1433

P1476

P2093

P2860

P304

P31

P356

P433

P478

P50

P577

P5875

P698

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P1433

P1476

P2093

P2860

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P433

P478

P50

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P932