Skip to main content
. 2023 Dec 8;13:44. doi: 10.5334/tohm.825

Table 3.

TOR2A variants reported by NCBI’s ClinVar and PubMed.


VARIANT(ACCESSION) PROTEIN CHANGE CONDITION (NUMBER OF PROBANDS) CLINICAL SIGNIFICANCE gnomAD V3.1.2 (ALLELE FREQUENCY) CADDPHRED-SCALED MetaLR REVEL REF.

c.194T>C
(SCV003669886.1)
p. Leu65Pro Inborn genetic disease
(N = 1)
Uncertain significance 31.0 0.2864 0.664 [14]

c.247C>T
(SCV003546031.1)
p. Pro83Ser Inborn genetic disease
(N = 1)
Uncertain significance 2/152200 28.3 0.2573 0.376

c.338G>A
(SCV003885945.1)
p. Gly113Asp Inborn genetic disease
(N = 1)
Uncertain significance 31.0 0.02109 0.024

c.423T>A
(SCV003950745.1)
p. Asp141Glu Inborn genetic disease
(N = 1)
Uncertain significance 2/152214 16.1 0.04569 0.031

c.463C>T
(SCV003757814.1)
p. Arg155Cys Inborn genetic disease
(N = 1)
Uncertain significance 2/152230 24.5 0.3835 0.281

c.553T>C
(SCV003661212.1)
p. Tyr185His Inborn genetic disease
(N = 1)
Uncertain significance 23.5 0.0976 0.182

c.62T>C
(SCV003534402.1)
p.Val21Ala Inborn genetic disease
(N = 1)
Uncertain significance 3/152194 14.8 0.1089 0.0540

c.734A>G
(SCV003566928.1)
p.Asn245Ser Inborn genetic disease
(N = 1)
Uncertain significance 1/231144 7.8 0.0521 0.040

c.737C>T
(SCV003615110.1)
p.Ser246Leu Inborn genetic disease
(N = 1)
Uncertain significance 2/152226 26.0 0.4321 0.563

c.766G>A
(SCV004004774.1)
p. Ala256Thr Inborn genetic disease
(N = 1)
Uncertain significance 6/276576 21.2 0.1399 0.089

c.785C>G
(SCV003708033.1)
p. Pro262Arg Inborn genetic disease
(N = 1)
Uncertain significance 4/152198 27.3 0.5009 0.809

c.805C>T
(SCV003951769.1)
p. Arg269Trp Inborn genetic disease
(N = 1)
Uncertain significance 7/279546 26.5 0.3993 0.172

c.907C>A
(SCV001041175.1)
p. Gln303Lys Inborn genetic disease
(N = 1)
Uncertain significance 67/152230 17.1 0.0676 0.146

c.925G>A
(SCV003551979.1)
p. Gly309Ser Inborn genetic disease
(N = 1)
Uncertain significance 9/152230 27.1 0.5849 0.813

c.937G>A
(SCV003529445.1)
p. Val313Met Inborn genetic disease
(N = 1)
Uncertain significance 6/152246 25.4 0.5539 0.658

c.568C>T
(NM_001085347.3)
p.Arg190Cys BSP/BSP+ (N = 3, one pedigree) Likely pathogenic 5/152188 29.2 0.548 0.5 [11]

c.593+31G>C
(NM_001085347.2)
p. Trp208Cys BSP Benign 97582/ 152038 0.203 0.000 0.0320 [13]

c.-42G>A
(NM_130459.3)
NA BSP/BSP+ (N = 6) Benign 12139/152166 12.2 [12]

c.277_288dup (NM_001085347.3) p.Gly93_Gly96dup BSP/BSP+ (N = 1 homozygote) Uncertain significance 11/152226 21.5

c.418–51T>G
(NM_001085347.3)
NA BSP/BSP+ (N = 35), Controls (N = 40) Benign 98659/151956 10.3

c.555C>T
(NM_001085347.3)
p.Tyr185= BSP/BSP+ (N = 1), Controls (N = 0) Uncertain significance 9/152204 0.382

c.593+36del
(NM_001085347.3)
c.629del
(NM_130459.4)
NA
p.Gly210AlafsTer60
BSP/BSP+ (N = 1), Controls (N = 0) Uncertain significance 3/152204 0.81

c.594–46C>T
(NM_001085347.3)
NA BSP/BSP+ (N=23 heterozygotes, 6 homozygotes), Controls (18 heterozygotes, 8 homozygotes) Benign 18456/152198 0.252

c.721+32A>G
(NM_001085347.3)
NA BSP/BSP+ (N=35 heterozygotes, 9 homozygotes), Controls (48 heterozygotes, 24 homozygotes) Benign 101903/152022 4.89

c.721+52G>A
(NM_001085347.3)
NA BSP/BSP+ (N = 10), Controls (N = 12) Benign 25185/152088 7.55

c.594–59C>T (NM_001085347.3) NA BSP/BSP+ (N = 1), Controls (N = 0) Uncertain significance 7/152224 0.154

c.721+83C>T
(NM_001085347.3)
NA BSP/BSP+ (N = 1), Controls (N = 0) Uncertain significance 1.89

c.594-55C>A
(NM_001085347.3)
NA BSP/BSP+ (N = 1), Controls (N = 0) Uncertain significance 28/152228 6.55

c.*28del
(NM_001085347.3)
NA BSP/BSP+ (N = 9), Controls (N = 8) Benign 387/152220 0.128

c.*125A>G
(NM_001085347.3)
N/A BSP/BSP+ (N = 1), Controls (N = 1) Uncertain significance 1/152222 5.65 NA NA

c.786G>A
(NM_001085347.3)
p.Pro262= BSP/BSP+ (N = 1), Controls (N = 1) Benign 83/152120 0.572 0.0614 0.0440