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Penny Handford

This is a preview profile on BiomedExperts - the first literature-based scientific social network. It brings the right researchers together and allows them to collaborate online. Collexis and Dell provide the BiomedExperts network of +1.5 Million pre-calculated profiles free of charge to researchers worldwide.

Research Profile (preview)

Chemicals & Drugs

Disorders

Marfan Syndrome

Concepts & Ideas

Genes & Molecular Sequences

Amino Acid Sequence

Phenomena

Anatomy

Microfibrils

Physiology

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Network (preview)

Co-Publications
Name
21
Knott, Vroni
17
Downing, Kristina
12
Brownlee, George
11
Campbell, Iain
11
Whiteman, Pat
7
Hutchinson, Sarah
7
Werner, Joern
6
Redfield, Christina
4
Lea, Susan
4
Smallridge, Rachel
4
Kettle, Susan
4
Yuan, Xuemei
3
Roversi, Pietro
3
Firth, Helen
3
Halliday, Dorothy

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Publications

TOP 3
Jensen Sacha A; Iqbal Sarah; Lowe Edward D; Redfield Christina; Handford Penny A
Structure and interdomain interactions of a hybrid domain: a disulphide-rich module of the fibrillin/LTBP superfamily of matrix proteins.
Cordle Jemima; Johnson Steven; Tay Joyce Zi Yan; Roversi Pietro; Wilkin Marian B; de Madrid Beatriz Hernández; Shimizu Hideyuki; Jensen Sacha; Whiteman Pat; Jin Boquan; Redfield Christina; Baron Martin; Lea Susan M; Handford Penny A
A conserved face of the Jagged/Serrate DSL domain is involved in Notch trans-activation and cis-inhibition.
Jovanovic Jelena; Iqbal Sarah; Jensen Sacha; Mardon Helen; Handford Penny
Fibrillin-integrin interactions in health and disease.

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All Publications

2009: Jensen Sacha A; Iqbal Sarah; Lowe Edward D; Redfield Christina; Handford Penny A
Structure and interdomain interactions of a hybrid domain: a disulphide-rich module of the fibrillin/LTBP superfamily of matrix proteins.
Structure (London, England : 1993) 2009;17(5):759-68.
2008: Cordle Jemima; Johnson Steven; Tay Joyce Zi Yan; Roversi Pietro; Wilkin Marian B; de Madrid Beatriz Hernández; Shimizu Hideyuki; Jensen Sacha; Whiteman Pat; Jin Boquan; Redfield Christina; Baron Martin; Lea Susan M; Handford Penny A
A conserved face of the Jagged/Serrate DSL domain is involved in Notch trans-activation and cis-inhibition.
Nature structural & molecular biology 2008;15(8):849-57.
2008: Jovanovic Jelena; Iqbal Sarah; Jensen Sacha; Mardon Helen; Handford Penny
Fibrillin-integrin interactions in health and disease.
Biochemical Society transactions 2008;36(Pt 2):257-62.
2008: Cordle Jemima; Redfieldz Christina; Stacey Martin; van der Merwe P Anton; Willis Antony C; Champion Brian R; Hambleton Sophie; Handford Penny A
Localization of the delta-like-1-binding site in human Notch-1 and its modulation by calcium affinity.
The Journal of biological chemistry 2008;283(17):11785-93.
2007: Davis Jason A; Handford Penny A; Redfield Christina
The N1317H substitution associated with Leber congenital amaurosis results in impaired interdomain packing in human CRB1 epidermal growth factor-like (EGF) domains.
The Journal of biological chemistry 2007;282(39):28807-14.
2007: Abbott Rachel J M; Spendlove Ian; Roversi Pietro; Fitzgibbon Hannah; Knott Vroni; Teriete Peter; McDonnell James M; Handford Penny A; Lea Susan M
Structural and functional characterization of a novel T cell receptor co-regulatory protein complex, CD97-CD55.
The Journal of biological chemistry 2007;282(30):22023-32.
2007: Yu Jing; Tirlapur Uday; Fairbank Jeremy; Handford Penny; Roberts Sally; Winlove C Peter; Cui Zhanfeng; Urban Jill
Microfibrils, elastin fibres and collagen fibres in the human intervertebral disc and bovine tail disc.
Journal of anatomy 2007;210(4):460-71.
2007: Whiteman Pat; Willis Antony C; Warner Andrew; Brown James; Redfield Christina; Handford Penny A
Cellular and molecular studies of Marfan syndrome mutations identify co-operative protein folding in the cbEGF12-13 region of fibrillin-1.
Human molecular genetics 2007;16(8):907-18.
2007: Jovanovic Jelena; Takagi Junichi; Choulier Laurence; Abrescia Nicola G A; Stuart David I; van der Merwe P Anton; Mardon Helen J; Handford Penny A
alphaVbeta6 is a novel receptor for human fibrillin-1. Comparative studies of molecular determinants underlying integrin-rgd affinity and specificity.
The Journal of biological chemistry 2007;282(9):6743-51.
2006: Robinson P N; Arteaga-Solis E; Baldock C; Collod-Béroud G; Booms P; De Paepe A; Dietz H C; Guo G; Handford P A; Judge D P; Kielty C M; Loeys B; Milewicz D M; Ney A; Ramirez F; Reinhardt D P; Tiedemann K; Whiteman P; Godfrey M
The molecular genetics of Marfan syndrome and related disorders.
Journal of medical genetics 2006;43(10):769-87.
2006: Whiteman Pat; Hutchinson Sarah; Handford Penny A
Fibrillin-1 misfolding and disease.
Antioxidants & redox signaling 2006;8(3-4):338-46.
2005: Periz Javier; Gill Andrew C; Knott Vroni; Handford Penny A; Tomley Fiona M
Calcium binding activity of the epidermal growth factor-like domains of the apicomplexan microneme protein EtMIC4.
Molecular and biochemical parasitology 2005;143(2):192-9.
2005: Jensen Sacha A; Corbett Adam R; Knott Vroni; Redfield Christina; Handford Penny A
Ca2+-dependent interface formation in fibrillin-1.
The Journal of biological chemistry 2005;280(14):14076-84.
2005: Hutchinson Sarah; Aplin Robin T; Webb Heather; Kettle Susan; Timmermans Janneke; Boers Godfried H J; Handford Penny A
Molecular effects of homocysteine on cbEGF domain structure: insights into the pathogenesis of homocystinuria.
Journal of molecular biology 2005;346(3):833-44.
2004: Hambleton Sophie; Valeyev Najl V; Muranyi Andreas; Knott Vroni; Werner Jörn M; McMichael Andrew J; Handford Penny A; Downing A Kristina
Structural and functional properties of the human notch-1 ligand binding region.
Structure (London, England : 1993) 2004;12(12):2173-83.
2004: Suk Ji Young; Jensen Sacha; McGettrick Aileen; Willis Antony C; Whiteman Pat; Redfield Christina; Handford Penny A
Structural consequences of cysteine substitutions C1977Y and C1977R in calcium-binding epidermal growth factor-like domain 30 of human fibrillin-1.
The Journal of biological chemistry 2004;279(49):51258-65.
2004: den Hollander Anneke I; Davis Jason; van der Velde-Visser Saskia D; Zonneveld Marijke N; Pierrottet Chiara O; Koenekoop Robert K; Kellner Ulrich; van den Born L Ingeborgh; Heckenlively John R; Hoyng Carel B; Handford Penny A; Roepman Ronald; Cremers Frans P M
CRB1 mutation spectrum in inherited retinal dystrophies.
Human mutation 2004;24(5):355-69.
2004: Muranyi Andreas; Hambleton Sophie; Knott Vroni; McMichael Andrew; Handford Penny A; Downing A Kristina
1H, 13C, and 15N resonance assignments of human Notch-1 calcium binding EGF domains 11-13.
Journal of biomolecular NMR 2004;29(3):443-4.
2004: Cardy Caroline M; Maskell Nick A; Handford Penny A; Arnold Anthony G; Davies Robert J O; Morrison Patrick J; Thornley Peter E
Familial spontaneous pneumothorax and FBN1 mutations.
American journal of respiratory and critical care medicine 2004;169(11):1260-2.
2004: Abbott Rachel J M; Knott Vroni; Roversi Pietro; Neudeck Saskia; Lukacik Petra; Handford Penny A; Lea Susan M
Crystallization and preliminary X-ray diffraction analysis of three EGF domains of EMR2, a 7TM immune-system molecule.
Acta crystallographica. Section D, Biological crystallography 2004;60(Pt 5):936-8.
2004: Lee Stephen S J; Knott Vroni; Jovanovic Jelena; Harlos Karl; Grimes Jonathan M; Choulier Laurence; Mardon Helen J; Stuart David I; Handford Penny A
Structure of the integrin binding fragment from fibrillin-1 gives new insights into microfibril organization.
Structure (London, England : 1993) 2004;12(4):717-29.
2003: Lack Jeremy; O'Leary Joanne M; Knott Vroni; Yuan Xuemei; Rifkin Daniel B; Handford Penny A; Downing A Kristina
Solution structure of the third TB domain from LTBP1 provides insight into assembly of the large latent complex that sequesters latent TGF-beta.
Journal of molecular biology 2003;334(2):281-91.
2003: Hutchinson Sarah; Furger Andre; Halliday Dorothy; Judge Daniel P; Jefferson Andrew; Dietz Harry C; Firth Helen; Handford Penny A
Allelic variation in normal human FBN1 expression in a family with Marfan syndrome: a potential modifier of phenotype?
Human molecular genetics 2003;12(18):2269-76.
2003: Whiteman Pat; Handford Penny A
Defective secretion of recombinant fragments of fibrillin-1: implications of protein misfolding for the pathogenesis of Marfan syndrome and related disorders.
Human molecular genetics 2003;12(7):727-37.
2003: Smallridge Rachel S; Whiteman Pat; Werner Jörn M; Campbell Iain D; Handford Penny A; Downing A Kristina
Solution structure and dynamics of a calcium binding epidermal growth factor-like domain pair from the neonatal region of human fibrillin-1.
The Journal of biological chemistry 2003;278(14):12199-206.
2002: Halliday D J; Hutchinson S; Lonie L; Hurst J A; Firth H; Handford P A; Wordsworth P
Twelve novel FBN1 mutations in Marfan syndrome and Marfan related phenotypes test the feasibility of FBN1 mutation testing in clinical practice.
Journal of medical genetics 2002;39(8):589-93.
2002: Yuan Xuemei; Werner Jörn M; Lack Jeremy; Knott Vroni; Handford Penny A; Campbell Iain D; Downing A Kristina
Effects of the N2144S mutation on backbone dynamics of a TB-cbEGF domain pair from human fibrillin-1.
Journal of molecular biology 2002;316(1):113-25.
2001: Hutchinson S; Wordsworth B P; Handford P A
Marfan syndrome caused by a mutation in FBN1 that gives rise to cryptic splicing and a 33 nucleotide insertion in the coding sequence.
Human genetics 2001;109(4):416-20.
2001: Saha S; Boyd J; Werner J M; Knott V; Handford P A; Campbell I D; Downing A K
Solution structure of the LDL receptor EGF-AB pair: a paradigm for the assembly of tandem calcium binding EGF domains.
Structure (London, England : 1993) 2001;9(6):451-6.
2001: Lin H H; Stacey M; Saxby C; Knott V; Chaudhry Y; Evans D; Gordon S; McKnight A J; Handford P; Lea S
Molecular analysis of the epidermal growth factor-like short consensus repeat domain-mediated protein-protein interactions: dissection of the CD97-CD55 complex.
The Journal of biological chemistry 2001;276(26):24160-9.
2001: Whiteman P; Smallridge R S; Knott V; Cordle J J; Downing A K; Handford P A
A G1127S change in calcium-binding epidermal growth factor-like domain 13 of human fibrillin-1 causes short range conformational effects.
The Journal of biological chemistry 2001;276(20):17156-62.
2000: Handford P A
Fibrillin-1, a calcium binding protein of extracellular matrix.
Biochimica et biophysica acta 2000;1498(2-3):84-90.
2000: Handford P A; Downing A K; Reinhardt D P; Sakai L Y
Fibrillin: from domain structure to supramolecular assembly.
Matrix biology : journal of the International Society for Matrix Biology 2000;19(6):457-70.
2000: McGettrick A J; Knott V; Willis A; Handford P A
Molecular effects of calcium binding mutations in Marfan syndrome depend on domain context.
Human molecular genetics 2000;9(13):1987-94.
2000: Werner J M; Knott V; Handford P A; Campbell I D; Downing A K
Backbone dynamics of a cbEGF domain pair in the presence of calcium.
Journal of molecular biology 2000;296(4):1065-78.
2000: Kettle S; Card C M; Hutchinson S; Sykes B; Handford P A
Characterisation of fibrillin-1 cDNA clones in a human fibroblast cell line that assembles microfibrils.
The international journal of biochemistry & cell biology 2000;32(2):201-14.
1999: Halliday D; Hutchinson S; Kettle S; Firth H; Wordsworth P; Handford P A
Molecular analysis of eight mutations in FBN1.
Human genetics 1999;105(6):587-97.
1999: Smallridge R S; Whiteman P; Doering K; Handford P A; Downing A K
EGF-like domain calcium affinity modulated by N-terminal domain linkage in human fibrillin-1.
Journal of molecular biology 1999;286(3):661-8.
1999: Kettle S; Yuan X; Grundy G; Knott V; Downing A K; Handford P A
Defective calcium binding to fibrillin-1: consequence of an N2144S change for fibrillin-1 structure and function.
Journal of molecular biology 1999;285(3):1277-87.
1998: Whiteman P; Downing A K; Handford P A
NMR analysis of cbEGF domains gives new insights into the structural consequences of a P1148A substitution in fibrillin-1.
Protein engineering 1998;11(11):957-9.
1998: Yuan X; Werner J M; Knott V; Handford P A; Campbell I D; Downing K
Effects of proline cis-trans isomerization on TB domain secondary structure.
Protein science : a publication of the Protein Society 1998;7(10):2127-35.
1998: Whiteman P; Downing A K; Smallridge R; Winship P R; Handford P A
A Gly --> Ser change causes defective folding in vitro of calcium-binding epidermal growth factor-like domains from factor IX and fibrillin-1.
The Journal of biological chemistry 1998;273(14):7807-13.
1998: Cardy C M; Handford P A
Metal ion dependency of microfibrils supports a rod-like conformation for fibrillin-1 calcium-binding epidermal growth factor-like domains.
Journal of molecular biology 1998;276(5):855-60.
1997: Yuan X; Downing A K; Knott V; Handford P A
Solution structure of the transforming growth factor beta-binding protein-like module, a domain associated with matrix fibrils.
The EMBO journal 1997;16(22):6659-66.
1996: Downing A K; Knott V; Werner J M; Cardy C M; Campbell I D; Handford P A
Solution structure of a pair of calcium-binding epidermal growth factor-like domains: implications for the Marfan syndrome and other genetic disorders.
Cell 1996;85(4):597-605.
1996: Knott V; Downing A K; Cardy C M; Handford P
Calcium binding properties of an epidermal growth factor-like domain pair from human fibrillin-1.
Journal of molecular biology 1996;255(1):22-7.
1995: Rao Z; Handford P; Mayhew M; Knott V; Brownlee G G; Stuart D
The structure of a Ca(2+)-binding epidermal growth factor-like domain: its role in protein-protein interactions.
Cell 1995;82(1):131-41.
1995: Rao Z; Handford P A; Knott V; Mayhew M; Brownlee G G; Stuart D
Crystallization of a calcium-binding EGF-like domain.
Acta crystallographica. Section D, Biological crystallography 1995;51(Pt 3):402-3.
1995: Handford P; Downing A K; Rao Z; Hewett D R; Sykes B C; Kielty C M
The calcium binding properties and molecular organization of epidermal growth factor-like domains in human fibrillin-1.
The Journal of biological chemistry 1995;270(12):6751-6.
1994: Hughes P E; Handford P A; Austen D E; Brownlee G G
Protein engineering of the hydrophobic domain of human factor IX.
Protein engineering 1994;7(9):1121-7.
1994: Mayhew M; Handford P; Brownlee G G
The binding of natural variants of human factor IX to endothelial cells.
FEBS letters 1994;341(1):74-8.
1993: Sunnerhagen M S; Persson E; Dahlqvist I; Drakenberg T; Stenflo J; Mayhew M; Robin M; Handford P; Tilley J W; Campbell I D
The effect of aspartate hydroxylation on calcium binding to epidermal growth factor-like modules in coagulation factors IX and X.
The Journal of biological chemistry 1993;268(31):23339-44.
1993: Hughes P E; Morgan G; Rooney E K; Brownlee G G; Handford P
Tyrosine 69 of the first epidermal growth factor-like domain of human factor IX is essential for clotting activity.
The Journal of biological chemistry 1993;268(24):17727-33.
1992: Mayhew M; Handford P; Baron M; Tse A G; Campbell I D; Brownlee G G
Ligand requirements for Ca2+ binding to EGF-like domains.
Protein engineering 1992;5(6):489-94.
1992: Baron M; Norman D G; Harvey T S; Handford P A; Mayhew M; Tse A G; Brownlee G G; Campbell I D
The three-dimensional structure of the first EGF-like module of human factor IX: comparison with EGF and TGF-alpha.
Protein science : a publication of the Protein Society 1992;1(1):81-90.
1991: Handford P A; Mayhew M; Brownlee G G
Calcium binding to fibrillin?
Nature 1991;353(6343):395.
1991: Handford P A; Mayhew M; Baron M; Winship P R; Campbell I D; Brownlee G G
Key residues involved in calcium-binding motifs in EGF-like domains.
Nature 1991;351(6322):164-7.
1991: Handford P A; Winship P R; Brownlee G G
Protein engineering of the propeptide of human factor IX.
Protein engineering 1991;4(3):319-23.
1990: Handford P A; Baron M; Mayhew M; Willis A; Beesley T; Brownlee G G; Campbell I D
The first EGF-like domain from human factor IX contains a high-affinity calcium binding site.
The EMBO journal 1990;9(2):475-80.
1988: Rees D J; Jones I M; Handford P A; Walter S J; Esnouf M P; Smith K J; Brownlee G G
The role of beta-hydroxyaspartate and adjacent carboxylate residues in the first EGF domain of human factor IX.
The EMBO journal 1988;7(7):2053-61.

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