The structure of pathogenic huntingtin exon 1 defines the bases of its aggregation propensity.

Elena-Real CA, Sagar A, Urbanek A, Popovic M, Morató A, Estaña A, Fournet A, Doucet C, Lund XL, Shi ZD, Costa L, Thureau A, Allemand F, Swenson RE, Milhiet PE, Crehuet R, Barducci A, Cortés J, Sinnaeve D, Sibille N, Bernadó P
Nat Struct Mol Biol (2023 Mar 2)
PMID: 36864173
doi: 10.1038/s41594-023-00920-0
Submitted to SASBDB: 2022 Nov 30
Published in SASBDB:

SASDQR8 – Exon1 of Non pathogenic form of Huntingtin (H16) with GFP fused at the C-terminus

Huntingtin experimental SAS data
OTHER [STATIC IMAGE] model
Sample: Huntingtin monomer, 39 kDa Homo sapiens protein
Buffer: 20mM BisTris-HCl, 150mM NaCl, pH: 6.5
Experiment: SAXS data collected at SWING, SOLEIL on 2019 Dec 3
RgGuinier 3.3 nm
Dmax 16.0 nm
VolumePorod 63 nm3

SASDQS8 – Exon1 of Pathogenic form of Huntingtin (H46) with GFP fused at the C-terminus

Huntingtin experimental SAS data
OTHER [STATIC IMAGE] model
Sample: Huntingtin monomer, 43 kDa Homo sapiens protein
Buffer: 20mM BisTris-HCl, 150mM NaCl, pH: 6.5
Experiment: SAXS data collected at EMBL P12, PETRA III on 2019 Oct 28
RgGuinier 4.2 nm