Table 2 Predicted destabilization of PAH-associated mutants of BMPR2.

From: Structural consequences of BMPR2 kinase domain mutations causing pulmonary arterial hypertension

Class

Mutation site

Predicted ΔΔGa

1. Buried steric clashes

S301P

N.D.b

A313P

>10

C347R

>10

C347Y

>10

C420R

>10

C420Y

>10

C483R

>10

R491W

>10

2. Buried broken interactions

L401S

4.97

R419S

7.41

R491Q

5.94

3. Exposed

R266T

0.78

M273R

2.08

R303H

5.57

D485G

7.97

A490V

5.56

C496Y

4.93

E503D

1.66

  1. aStructural models and destabilization free energies were calculated using the Eris server (http://dokhlab.unc.edu/tools/eris/). The ΔΔG value defines the difference in the free energy between the wild-type and mutant structures. Values > 0 are destabilizing.
  2. bNot determined. The S301P mutation was too severe for the Eris server to model. The structural model was instead calculated using ICM27.
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