Collagen VI Microfibril Structure Reveals the Mechanism for Heterotrimerisation and the Clustering of Inherited Pathogenic Mutations

Mark H. Becker.

SASDWL2 – C-terminal mini-Collagen VI heterotrimer

Collagen alpha-1(VI) chain
Collagen alpha-2(VI) chain (R680H, K966N, Q967E)
Collagen alpha-3(VI) chain (D2357R, K2367R, D2431V, R2441T, R2609A, R2610A)
MWexperimental 156 kDa
MWexpected 159 kDa
VPorod 478 nm3
log I(s) 1.88×10-1 1.88×10-2 1.88×10-3 1.88×10-4
Collagen alpha-1(VI) chain Collagen alpha-2(VI) chain (R680H, K966N, Q967E) Collagen alpha-3(VI) chain (D2357R, K2367R, D2431V, R2441T, R2609A, R2610A) small angle scattering data  s, nm-1
ln I(s)
Collagen alpha-1(VI) chain Collagen alpha-2(VI) chain (R680H, K966N, Q967E) Collagen alpha-3(VI) chain (D2357R, K2367R, D2431V, R2441T, R2609A, R2610A) Guinier plot ln 1.89×10-1 Rg: 4.8 nm 0 (4.8 nm)-2 s2
(sRg)2I(s)/I(0)
Collagen alpha-1(VI) chain Collagen alpha-2(VI) chain (R680H, K966N, Q967E) Collagen alpha-3(VI) chain (D2357R, K2367R, D2431V, R2441T, R2609A, R2610A) Kratky plot 1.104 0 3 sRg
p(r)
Collagen alpha-1(VI) chain Collagen alpha-2(VI) chain (R680H, K966N, Q967E) Collagen alpha-3(VI) chain (D2357R, K2367R, D2431V, R2441T, R2609A, R2610A) pair distance distribution function Rg: 4.8 nm 0 Dmax: 18.7 nm

Data validation


Fits and models


log I(s)
 s, nm-1
Collagen alpha-1(VI) chain Collagen alpha-2(VI) chain (R680H, K966N, Q967E) Collagen alpha-3(VI) chain (D2357R, K2367R, D2431V, R2441T, R2609A, R2610A) ALPHAFOLD model

Synchrotron SAXS data from solutions of the C-terminal mini-Collagen VI heterotrimer in Tris-buffered saline, pH 7.4 were collected on the B21 beam line at the Diamond Light Source (Didcot, UK) using a Eiger 4M detector at a sample-detector distance of 3.7 m and at a wavelength of λ = 0.09537 nm (I(s) vs s, where s = 4πsinθ/λ, and 2θ is the scattering angle). One solute concentration of 1.00 mg/ml was measured at 20°C. 20 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.

CAUTION: Likely aggregated.

Collagen alpha-1(VI) chain (ColVIa1)
Mol. type   Protein
Organism   Homo sapiens
Olig. state   Other
Mon. MW   52.2 kDa
 
UniProt   P12109 (566-1028)
Sequence   FASTA
 
Collagen alpha-2(VI) chain (R680H, K966N, Q967E) (ColVIa2)
Mol. type   Protein
Organism   Homo sapiens
Olig. state   Other
Mon. MW   52.5 kDa
 
UniProt   P12110 (564-1019)
Sequence   FASTA
 
Collagen alpha-3(VI) chain (D2357R, K2367R, D2431V, R2441T, R2609A, R2610A) (ColVIa3)
Mol. type   Protein
Organism   Homo sapiens
Olig. state   Other
Mon. MW   54.2 kDa
 
UniProt   P12111 (2347-2820)
Sequence   FASTA