Table I

Comparison of the mutations modeled showing structural differences between each of the disease-associated mutations

Columns are as follows. POS, identity of mutation and position along the chain. OI, osteogenesis imperfecta clinical phenotype. r.m.s.d., average r.m.s.d. of wild type peptides (standard deviation). r.m.s.d. MT, average r.m.s.d. of mutant peptides (standard deviation). HB, all hydrogen bonds between main chain atoms in wild type peptides. HB MT, all hydrogen bonds between main chain atoms in mutant peptides. SHB, all solvent-solute hydrogen bonds between main chain atoms in wild type peptides. SHB MT, all solvent hydrogen bonds between main chain atoms in mutant peptides.

POSOIar.m.s.d.r.m.s.d. MTHBbHB MTSHBSHB MT
G910A*1.33 (0.16)0.80 (0.14)161511
G928A*1.10 (0.13)0.66 (0.08)161559
G256V*1.36 (0.16)0.69 (0.07)171213
G586V*1.63 (0.15)1.41 (0.39)121233
G637V*1.59 (0.20)0.77 (0.13)141234
G802V*1.44 (0.23)0.72 (0.11)161366
G844V*1.58 (0.18)0.89 (0.20)151209
G973V*2.01 (0.44)0.86 (0.12)171223
G451S*1.22 (0.17)0.73 (0.13)161312
G478S*1.14 (0.15)0.77 (0.15)151455
G565S*1.33 (0.15)0.76 (0.12)161634
G598S*1.23 (0.15)0.71 (0.09)151411
G631S*1.22 (0.16)0.72 (0.10)161326
G913S**1.25 (0.13)0.68 (0.09)161533
G964S*1.39 (0.13)0.75 (0.09)161203
G691C*1.69 (0.26)0.71 (0.09)161361
G718C*1.33 (0.11)0.69 (0.09)161420
G748C*1.17 (0.12)0.80 (0.12)161423
G244C*1.27 (0.10)0.59 (0.06)161515
G448C*1.93 (0.20)0.73 (0.10)161501
G904C*1.06 (0.11)0.73 (0.10)161400
G946C*1.22 (0.12)0.71 (0.10)161532
G988C*1.27 (0.20)0.77 (0.11)151329
G85VI1.31 (0.13)1.18 (0.26)1511310
G901SI1.25 (0.11)0.63 (0.07)151501
G43CI1.23 (0.16)0.72 (0.11)181612
G46CI1.31 (0.16)0.74 (0.12)171711
G94CI1.64 (0.25)0.95 (0.14)141344
G205CI1.60 (0.25)0.75 (0.13)161400
G223CI, IV1.01 (0.08)0.87 (0.11)151636
G154AIII1.21 (0.23)0.91 (0.17)171505
G247S*,III1.16 (0.15)0.75 (0.13)161411
G541SIII1.36 (0.13)0.76 (0.13)151754
G589SIII, IV1.35 (0.10)0.70 (0.09)161402
G601SIII, IV1.58 (0.26)0.73 (0.12)161447
G643SIII1.00 (0.10)0.85 (0.23)161475
G661SIII1.40 (0.16)0.79 (0.13)161511
G415S*, III, IV1.40 (0.16)0.90 (0.18)161457
G844SIII1.01 (0.09)0.72 (0.08)161446
G862SIII, *1.19 (0.12)0.73 (0.11)151165
G871SIII1.08 (0.12)0.75 (0.11)161500
G898SIII1.36 (0.20)0.74 (0.11)161423
G973SIII1.19 (0.20)0.81 (0.16)171410
G175CIII, IV1.16 (0.19)0.69 (0.08)161638
G211CIII, IV2.10 (0.34)1.27 (0.19)141302
G226CIII, IV1.15 (0.20)0.68 (0.10)161653
G415CIII, IV1.42 (0.21)0.90 (0.21)141644
G526CIII1.30 (0.16)0.80 (0.17)181835
G868CIII1.31 (0.19)0.70 (0.09)161321
G220AIV1.04 (0.18)0.71 (0.08)161454
G382SIV1.28 (0.14)0.76 (0.10)151130
G448SIV1.50 (0.21)0.77 (0.17)171642
G832SIV1.04 (0.17)0.75 (0.09)141355
G880SIV1.15 (0.09)0.64 (0.07)161520
G178CIV1.36 (0.18)0.70 (0.09)161622
G349CIV1.30 (0.15)0.83 (0.22)151634
G382CIV1.49 (0.29)1.45 (0.53)151031
G523CIV1.62 (0.28)0.76 (0.10)191711
  • a Lethal OI (Type II) is represented by an asterisk.

  • b All hydrogen bond counts are those present greater than 80% of the time.