Critical Structural Defects Explain Filamin A Mutations Causing Mitral Valve Dysplasia.

Haataja TJK, Capoulade R, Lecointe S, Hellman M, Merot J, Permi P, Pentikäinen U, Biophys J 117(8):1467-1475 (2019) Europe PMC

SASDFE3 – Filamin A Ig-like domains 4-6, V711D mutant (FLNa4-6 V711D) at 4 mg/ml

Filamin A Ig-like domains 4-6, V711D mutation
MWexperimental 30 kDa
MWexpected 32 kDa
VPorod 64 nm3
log I(s) 3.07×101 3.07×100 3.07×10-1 3.07×10-2
Filamin A Ig-like domains 4-6, V711D mutation small angle scattering data  s, nm-1
ln I(s)
Filamin A Ig-like domains 4-6, V711D mutation Guinier plot ln 3.08×101 Rg: 4.1 nm 0 (4.1 nm)-2 s2
(sRg)2I(s)/I(0)
Filamin A Ig-like domains 4-6, V711D mutation Kratky plot 1.104 0 3 sRg
p(r)
Filamin A Ig-like domains 4-6, V711D mutation pair distance distribution function Rg: 4.2 nm 0 Dmax: 15 nm

Data validation


There are no models related to this curve.

Synchrotron SAXS data from solutions of Filamin A Ig-like domains 4-6 (V711D mutant) in 20 mM Tris, 100 mM NaCl, 1 mM DTT, pH 8 were collected on the BM29 beam line at the ESRF (Grenoble, France) using a Pilatus 1M detector at a sample-detector distance of 2.9 m and at a wavelength of λ = 0.1 nm (l(s) vs s, where s = 4πsinθ/λ, and 2θ is the scattering angle). One solute concentration of 4.00 mg/ml was measured at 20°C. 10 successive 1 second frames were collected. The data were normalized to the intensity of the transmitted beam and radially averaged; the scattering of the solvent-blank was subtracted.

Three-domain fragment of human Filamin A Ig-like domains 4-6 with V711D mutation (FLNa4-6 V711D) . There are two extra amino acids, Met and Ser, in the very N-terminus of the polypeptide that have resulted from TEV-cleavage of the GST fusion tag during the protein purification.

Filamin A Ig-like domains 4-6, V711D mutation (FLNa4-6 V711D)
Mol. type   Protein
Organism   Homo sapiens
Olig. state   Monomer
Mon. MW   31.5 kDa
 
UniProt   P21333 (574-869)
Sequence   FASTA