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PDBsum entry 1emo
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Matrix protein
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PDB id
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1emo
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Contents |
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* Residue conservation analysis
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DOI no:
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Cell
85:597-605
(1996)
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PubMed id:
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Solution structure of a pair of calcium-binding epidermal growth factor-like domains: implications for the Marfan syndrome and other genetic disorders.
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A.K.Downing,
V.Knott,
J.M.Werner,
C.M.Cardy,
I.D.Campbell,
P.A.Handford.
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ABSTRACT
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The nuclear magnetic resonance structure of a covalently linked pair of
calcium-binding (cb) epidermal growth factor-like (EGF) domains from human
fibrillin-1, the protein defective in the Marfan syndrome, is described. The two
domains are in a rigid, rod-like arrangement, stabilized by interdomain calcium
binding and hydrophobic interactions. We propose a model for the arrangement of
fibrillin monomers in microfibrils that reconciles structural and antibody
binding data, and we describe a set of disease-causing mutations that provide
the first clues to the specificity of cbEFG interactions. The residues involved
in stabilizing the domain linkage are highly conserved in fibrillin, fibulin,
thrombomodulin, and the low density lipoprotein receptor. We propose that the
relative orientation of tandem cbEGF domains in these proteins is similar, but
that in others, including Notch, pairs adopt a completely different conformation.
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Selected figure(s)
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Figure 2.
Figure 2. Schematic Illustration of the Domain–Domain
Packing Interactions of the fib32–33 PairTyr 2157 is
highlighted in yellow, and Ile 2185 and Gly 2186 are in cyan.
β-strands are depicted by green arrows, and calcium atoms are
shown in red. This figure was rendered ([38]) from MOLSCRIPT (
[34]) input.
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Figure 6.
Figure 6. The Domain Structure of Human Fibrillin-1 and a
Model for the Organization of Fibrillin Monomers within
Connective Tissue MicrofibrilsThe domain structure of human
fibrillin-1 ([41]) is shown on top, and the model for the
organization of fibrillin monomers within connective tissue
microfibrils is shown below. The position of the cbEGF domains
(32–36) shown in Figure 6 is highlighted. In the model,
fibrillin monomers are shown as arrows. The monomers are
arranged in a parallel, staggered, head-to-tail arrangement,
with N- and C-termini in the “bead” regions (shaded). The
dimensions of the fib32–33 pair, combined with the results of
antibody labeling data ( [46]), suggest that the extended
fibrillin monomer spans two interbead regions with 50% overlap,
as shown. The inverted Ys denote the position within each
monomer of a putative antibody binding site.
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The above figures are
reprinted
by permission from Cell Press:
Cell
(1996,
85,
597-605)
copyright 1996.
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Figures were
selected
by an automated process.
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Literature references that cite this PDB file's key reference
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PubMed id
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Reference
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M.A.Atanasova,
E.I.Konova,
T.A.Aleksovska,
K.N.Todorova,
M.N.Georgieva,
and
T.H.Lukanov
(2011).
Anti-fibrillin-1 autoantibodies in normal pregnancy and recurrent pregnancy loss.
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Autoimmun Rev,
10,
131-136.
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F.Ramirez,
and
L.Y.Sakai
(2010).
Biogenesis and function of fibrillin assemblies.
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Cell Tissue Res,
339,
71-82.
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H.Jafar-Nejad,
J.Leonardi,
and
R.Fernandez-Valdivia
(2010).
Role of glycans and glycosyltransferases in the regulation of Notch signaling.
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Glycobiology,
20,
931-949.
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I.Robertson,
S.Jensen,
and
P.Handford
(2010).
TB domain proteins: evolutionary insights into the multifaceted roles of fibrillins and LTBPs.
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Biochem J,
433,
263-276.
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N.Sharma,
A.M.Mali,
and
S.A.Bapat
(2010).
Spectrum of CREBBP mutations in Indian patients with Rubinstein-Taybi syndrome.
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J Biosci,
35,
187-202.
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R.Oberauer,
W.Rist,
M.C.Lenter,
B.S.Hamilton,
and
H.Neubauer
(2010).
EGFL6 is increasingly expressed in human obesity and promotes proliferation of adipose tissue-derived stromal vascular cells.
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Mol Cell Biochem,
343,
257-269.
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R.P.Jones,
C.Ridley,
T.A.Jowitt,
M.C.Wang,
M.Howard,
N.Bobola,
T.Wang,
P.N.Bishop,
C.M.Kielty,
C.Baldock,
A.J.Lotery,
and
D.Trump
(2010).
Structural effects of fibulin 5 missense mutations associated with age-related macular degeneration and cutis laxa.
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Invest Ophthalmol Vis Sci,
51,
2356-2362.
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Y.Hilhorst-Hofstee,
M.E.Rijlaarsdam,
A.J.Scholte,
M.Swart-van den Berg,
M.I.Versteegh,
I.van der Schoot-van Velzen,
H.J.Schäbitz,
E.K.Bijlsma,
M.J.Baars,
W.S.Kerstjens-Frederikse,
J.C.Giltay,
B.C.Hamel,
M.H.Breuning,
and
G.Pals
(2010).
The clinical spectrum of missense mutations of the first aspartic acid of cbEGF-like domains in fibrillin-1 including a recessive family.
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Hum Mutat,
31,
E1915-E1927.
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C.Guarnaccia,
S.Dhir,
A.Pintar,
and
S.Pongor
(2009).
The tetralogy of Fallot-associated G274D mutation impairs folding of the second epidermal growth factor repeat in Jagged-1.
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FEBS J,
276,
6247-6257.
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C.Opherk,
M.Duering,
N.Peters,
A.Karpinska,
S.Rosner,
E.Schneider,
B.Bader,
A.Giese,
and
M.Dichgans
(2009).
CADASIL mutations enhance spontaneous multimerization of NOTCH3.
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Hum Mol Genet,
18,
2761-2767.
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C.Stheneur,
G.Collod-Béroud,
L.Faivre,
J.F.Buyck,
L.Gouya,
J.M.Le Parc,
B.Moura,
C.Muti,
B.Grandchamp,
G.Sultan,
M.Claustres,
P.Aegerter,
B.Chevallier,
G.Jondeau,
and
C.Boileau
(2009).
Identification of the minimal combination of clinical features in probands for efficient mutation detection in the FBN1 gene.
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Eur J Hum Genet,
17,
1121-1128.
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D.Hubmacher,
and
D.P.Reinhardt
(2009).
One more piece in the fibrillin puzzle.
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Structure,
17,
635-636.
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L.Faivre,
G.Collod-Beroud,
B.Callewaert,
A.Child,
B.L.Loeys,
C.Binquet,
E.Gautier,
E.Arbustini,
K.Mayer,
M.Arslan-Kirchner,
A.Kiotsekoglou,
P.Comeglio,
M.Grasso,
C.Beroud,
C.Bonithon-Kopp,
M.Claustres,
C.Stheneur,
O.Bouchot,
J.E.Wolf,
P.N.Robinson,
L.Adès,
J.De Backer,
P.Coucke,
U.Francke,
A.De Paepe,
C.Boileau,
and
G.Jondeau
(2009).
Pathogenic FBN1 mutations in 146 adults not meeting clinical diagnostic criteria for Marfan syndrome: further delineation of type 1 fibrillinopathies and focus on patients with an isolated major criterion.
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Am J Med Genet A,
149,
854-860.
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L.Faivre,
G.Collod-Beroud,
B.Callewaert,
A.Child,
C.Binquet,
E.Gautier,
B.L.Loeys,
E.Arbustini,
K.Mayer,
M.Arslan-Kirchner,
C.Stheneur,
A.Kiotsekoglou,
P.Comeglio,
N.Marziliano,
J.E.Wolf,
O.Bouchot,
P.Khau-Van-Kien,
C.Beroud,
M.Claustres,
C.Bonithon-Kopp,
P.N.Robinson,
L.Adès,
J.De Backer,
P.Coucke,
U.Francke,
A.De Paepe,
G.Jondeau,
and
C.Boileau
(2009).
Clinical and mutation-type analysis from an international series of 198 probands with a pathogenic FBN1 exons 24-32 mutation.
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Eur J Hum Genet,
17,
491-501.
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L.Sabatier,
D.Chen,
C.Fagotto-Kaufmann,
D.Hubmacher,
M.D.McKee,
D.S.Annis,
D.F.Mosher,
and
D.P.Reinhardt
(2009).
Fibrillin assembly requires fibronectin.
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Mol Biol Cell,
20,
846-858.
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M.Ali,
M.McKibbin,
A.Booth,
D.A.Parry,
P.Jain,
S.A.Riazuddin,
J.F.Hejtmancik,
S.N.Khan,
S.Firasat,
M.Shires,
D.F.Gilmour,
K.Towns,
A.L.Murphy,
D.Azmanov,
I.Tournev,
S.Cherninkova,
H.Jafri,
Y.Raashid,
C.Toomes,
J.Craig,
D.A.Mackey,
L.Kalaydjieva,
S.Riazuddin,
and
C.F.Inglehearn
(2009).
Null mutations in LTBP2 cause primary congenital glaucoma.
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Am J Hum Genet,
84,
664-671.
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M.J.Sherratt
(2009).
Tissue elasticity and the ageing elastic fibre.
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Age (Dordr),
31,
305-325.
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M.Y.Frédéric,
C.Monino,
C.Marschall,
D.Hamroun,
L.Faivre,
G.Jondeau,
H.G.Klein,
L.Neumann,
E.Gautier,
C.Binquet,
C.Maslen,
M.Godfrey,
P.Gupta,
D.Milewicz,
C.Boileau,
M.Claustres,
C.Béroud,
and
G.Collod-Béroud
(2009).
The FBN2 gene: new mutations, locus-specific database (Universal Mutation Database FBN2), and genotype-phenotype correlations.
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Hum Mutat,
30,
181-190.
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M.Y.Frédéric,
M.Lalande,
C.Boileau,
D.Hamroun,
M.Claustres,
C.Béroud,
and
G.Collod-Béroud
(2009).
UMD-predictor, a new prediction tool for nucleotide substitution pathogenicity -- application to four genes: FBN1, FBN2, TGFBR1, and TGFBR2.
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Hum Mutat,
30,
952-959.
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N.Suda,
M.Shiga,
G.Ganburged,
and
K.Moriyama
(2009).
Marfan syndrome and its disorder in periodontal tissues.
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J Exp Zoolog B Mol Dev Evol,
312,
503-509.
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R.P.Jones,
M.C.Wang,
T.A.Jowitt,
C.Ridley,
K.T.Mellody,
M.Howard,
T.Wang,
P.N.Bishop,
A.J.Lotery,
C.M.Kielty,
C.Baldock,
and
D.Trump
(2009).
Fibulin 5 forms a compact dimer in physiological solutions.
|
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J Biol Chem,
284,
25938-25943.
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S.A.Jensen,
S.Iqbal,
E.D.Lowe,
C.Redfield,
and
P.A.Handford
(2009).
Structure and interdomain interactions of a hybrid domain: a disulphide-rich module of the fibrillin/LTBP superfamily of matrix proteins.
|
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Structure,
17,
759-768.
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PDB code:
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A.C.Tien,
A.Rajan,
K.L.Schulze,
H.D.Ryoo,
M.Acar,
H.Steller,
and
H.J.Bellen
(2008).
Ero1L, a thiol oxidase, is required for Notch signaling through cysteine bridge formation of the Lin12-Notch repeats in Drosophila melanogaster.
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J Cell Biol,
182,
1113-1125.
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C.F.Tu,
Y.T.Yan,
S.Y.Wu,
B.Djoko,
M.T.Tsai,
C.J.Cheng,
and
R.B.Yang
(2008).
Domain and functional analysis of a novel platelet-endothelial cell surface protein, SCUBE1.
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J Biol Chem,
283,
12478-12488.
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D.Hubmacher,
E.I.El-Hallous,
V.Nelea,
M.T.Kaartinen,
E.R.Lee,
and
D.P.Reinhardt
(2008).
Biogenesis of extracellular microfibrils: Multimerization of the fibrillin-1 C terminus into bead-like structures enables self-assembly.
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Proc Natl Acad Sci U S A,
105,
6548-6553.
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D.Li,
J.Yu,
F.Gu,
X.Pang,
X.Ma,
R.Li,
N.Liu,
and
X.Ma
(2008).
The roles of two novel FBN1 gene mutations in the genotype-phenotype correlations of Marfan syndrome and ectopia lentis patients with marfanoid habitus.
|
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Genet Test,
12,
325-330.
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J.Cordle,
C.Redfieldz,
M.Stacey,
P.A.van der Merwe,
A.C.Willis,
B.R.Champion,
S.Hambleton,
and
P.A.Handford
(2008).
Localization of the delta-like-1-binding site in human Notch-1 and its modulation by calcium affinity.
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J Biol Chem,
283,
11785-11793.
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S.A.Cain,
A.K.Baldwin,
Y.Mahalingam,
B.Raynal,
T.A.Jowitt,
C.A.Shuttleworth,
J.R.Couchman,
and
C.M.Kielty
(2008).
Heparan Sulfate Regulates Fibrillin-1 N- and C-terminal Interactions.
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J Biol Chem,
283,
27017-27027.
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V.B.Carruthers,
and
F.M.Tomley
(2008).
Microneme proteins in apicomplexans.
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Subcell Biochem,
47,
33-45.
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C.L.Kuo,
Z.Isogai,
D.R.Keene,
N.Hazeki,
R.N.Ono,
G.Sengle,
H.Peter Bächinger,
and
L.Y.Sakai
(2007).
Effects of fibrillin-1 degradation on microfibril ultrastructure.
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J Biol Chem,
282,
4007-4020.
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E.El-Hallous,
T.Sasaki,
D.Hubmacher,
M.Getie,
K.Tiedemann,
J.Brinckmann,
B.Bätge,
E.C.Davis,
and
D.P.Reinhardt
(2007).
Fibrillin-1 interactions with fibulins depend on the first hybrid domain and provide an adaptor function to tropoelastin.
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J Biol Chem,
282,
8935-8946.
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J.A.Davis,
P.A.Handford,
and
C.Redfield
(2007).
The N1317H substitution associated with Leber congenital amaurosis results in impaired interdomain packing in human CRB1 epidermal growth factor-like (EGF) domains.
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J Biol Chem,
282,
28807-28814.
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J.E.Wagenseil,
and
R.P.Mecham
(2007).
New insights into elastic fiber assembly.
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Birth Defects Res C Embryo Today,
81,
229-240.
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L.Faivre,
G.Collod-Beroud,
B.L.Loeys,
A.Child,
C.Binquet,
E.Gautier,
B.Callewaert,
E.Arbustini,
K.Mayer,
M.Arslan-Kirchner,
A.Kiotsekoglou,
P.Comeglio,
N.Marziliano,
H.C.Dietz,
D.Halliday,
C.Beroud,
C.Bonithon-Kopp,
M.Claustres,
C.Muti,
H.Plauchu,
P.N.Robinson,
L.C.Adès,
A.Biggin,
B.Benetts,
M.Brett,
K.J.Holman,
J.De Backer,
P.Coucke,
U.Francke,
A.De Paepe,
G.Jondeau,
and
C.Boileau
(2007).
Effect of mutation type and location on clinical outcome in 1,013 probands with Marfan syndrome or related phenotypes and FBN1 mutations: an international study.
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Am J Hum Genet,
81,
454-466.
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M.Tekin,
F.B.Cengiz,
E.Ayberkin,
T.Kendirli,
S.Fitoz,
E.Tutar,
E.Ciftçi,
and
A.Conba
(2007).
Familial neonatal Marfan syndrome due to parental mosaicism of a missense mutation in the FBN1 gene.
|
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Am J Med Genet A,
143,
875-880.
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N.Kobayashi,
G.Kostka,
J.H.Garbe,
D.R.Keene,
H.P.Bächinger,
F.G.Hanisch,
D.Markova,
T.Tsuda,
R.Timpl,
M.L.Chu,
and
T.Sasaki
(2007).
A comparative analysis of the fibulin protein family. Biochemical characterization, binding interactions, and tissue localization.
|
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J Biol Chem,
282,
11805-11816.
|
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S.F.Cummins,
F.Xie,
M.R.de Vries,
S.P.Annangudi,
M.Misra,
B.M.Degnan,
J.V.Sweedler,
G.T.Nagle,
and
C.H.Schein
(2007).
Aplysia temptin - the 'glue' in the water-borne attractin pheromone complex.
|
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FEBS J,
274,
5425-5437.
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S.L.Rasmussen,
L.Z.Holland,
M.Schubert,
L.Beaster-Jones,
and
N.D.Holland
(2007).
Amphioxus AmphiDelta: evolution of Delta protein structure, segmentation, and neurogenesis.
|
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Genesis,
45,
113-122.
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U.Ryde
(2007).
Accurate metal-site structures in proteins obtained by combining experimental data and quantum chemistry.
|
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Dalton Trans,
(),
607-625.
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C.Baldock,
V.Siegler,
D.V.Bax,
S.A.Cain,
K.T.Mellody,
A.Marson,
J.L.Haston,
R.Berry,
M.C.Wang,
J.G.Grossmann,
M.Roessle,
C.M.Kielty,
and
T.J.Wess
(2006).
Nanostructure of fibrillin-1 reveals compact conformation of EGF arrays and mechanism for extensibility.
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Proc Natl Acad Sci U S A,
103,
11922-11927.
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G.Ge,
and
D.S.Greenspan
(2006).
Developmental roles of the BMP1/TLD metalloproteinases.
|
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Birth Defects Res C Embryo Today,
78,
47-68.
|
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I.Bernascone,
S.Vavassori,
A.Di Pentima,
S.Santambrogio,
G.Lamorte,
A.Amoroso,
F.Scolari,
G.M.Ghiggeri,
G.Casari,
R.Polishchuk,
and
L.Rampoldi
(2006).
Defective intracellular trafficking of uromodulin mutant isoforms.
|
| |
Traffic,
7,
1567-1579.
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K.T.Mellody,
L.J.Freeman,
C.Baldock,
T.A.Jowitt,
V.Siegler,
B.D.Raynal,
S.A.Cain,
T.J.Wess,
C.A.Shuttleworth,
and
C.M.Kielty
(2006).
Marfan syndrome-causing mutations in fibrillin-1 result in gross morphological alterations and highlight the structural importance of the second hybrid domain.
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J Biol Chem,
281,
31854-31862.
|
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L.Tjeldhorn,
S.Rand-Hendriksen,
K.Gervin,
K.Brandal,
E.Inderhaug,
O.Geiran,
and
B.Paus
(2006).
Rapid and efficient FBN1 mutation detection using automated sample preparation and direct sequencing as the primary strategy.
|
| |
Genet Test,
10,
258-264.
|
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P.N.Robinson,
E.Arteaga-Solis,
C.Baldock,
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