All variants in the OTC gene

Information The variants shown are described using the NM_000531.5 transcript reference sequence.

666 entries on 7 pages. Showing entries 1 - 100.
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Effect     

Exon     

AscendingDNA change (cDNA)     

RNA change     

Protein     

Classification method     

Clinical classification     

DNA change (genomic) (hg19)     

DNA change (hg38)     

Published as     

ISCN     

DB-ID     

Variant remarks     

Reference     

ClinVar ID     

dbSNP ID     

Origin     

Segregation     

Frequency     

Re-site     

VIP     

Methylation     

Owner     
+/. _1_10_ c.-214_*368{0} r.0 p.0 - pathogenic g.(?_g.38211736)_(38280703_?)del g.(?_38352483)_(38421450_?)del delOTC gene >1.6 Mb - CYBB_000005 - PubMed: Climent 1999, PubMed: Martin-Hernandez 2014 - - Germline - - - - - Johan den Dunnen
+/. _1_10_ c.-214_*368{0} r.0 p.0 - pathogenic g.(?_g.38211736)_(38280703_?)del g.(?_38352483)_(38421450_?)del delOTC gene >1.6 Mb - CYBB_000005 - PubMed: Martin-Hernandez 2014 - - Germline - - - - - Johan den Dunnen
+/. _1_10_ c.-214_*368{0} r.0 p.0 - pathogenic g.(?_g.38211736)_(38280703_?)del g.(?_38352483)_(38421450_?)del del ex1-10 - CYBB_000005 - PubMed: Silvera-Ruiz 2019 - - Germline - 5/24 case chromosomes - - - Johan den Dunnen
+/. _1_10_ c.[-214_(1005+1_1006-1){2};(1005+1_1006-1)*368{0}] r.? p.? - pathogenic g.[(?_g.38211736)_(38271253_38280275)dup;(38271253_38280275)_(38280703_?)del] g.[(?_38352483)_(38412000_38421022)dup;(38412000_38421022)-(38421450_?)del] dup ex1-9/del ex10 - OTC_000442 - PubMed: Silvera-Ruiz 2019 - - Germline - 1/24 case chromosomes - - - Johan den Dunnen
+/. _1_10_ c.-2802714_*618720del r.0 p.0 - pathogenic (recessive) g.35409236_38899055del - hg17 35168893-38655271del - CYBB_000481 deletion incl. XK, CYBB, RPGR, OTC, TSPAN7 PubMed: Yamada 2010 - - De novo - - - - - Johan den Dunnen
?/. _1 c.-9484G>T r.(?) p.(=) - VUS g.38202466G>T g.38343213G>T - - OTC_000449 - PubMed: Yang 2018 - - Germline - - - - - Johan den Dunnen
+/. _1 c.-9484G>T r.(?) p.(=) - NA g.38202466G>T g.38343213G>T - - OTC_000449 in vitro expression analysis shows reduced reporter gene expression (0.2 in HuH-7 cells) PubMed: Yang 2018 - - Germline - - - - - Johan den Dunnen
?/. 1 c.-515_-512del r.(?) p.(?) - VUS g.38211435_38211438del g.38352182_38352185del -512insTCTT - OTC_000257 - PubMed: Azevedo 2003 - rs57752938 Germline - 2/36 MboII - - Johan den Dunnen
?/. 1 c.-515_-512del r.(?) p.(?) - VUS g.38211435_38211438del g.38352182_38352185del -512insTCTT - OTC_000257 - PubMed: Azevedo 2003 - rs57752938 Germline - 16/103 MboII - - Johan den Dunnen
?/. 1 c.-515_-512del r.(?) p.(?) - VUS g.38211435_38211438del g.38352182_38352185del -512insTCTT - OTC_000257 - PubMed: Azevedo 2003 - rs57752938 Germline - 1/57 MboII - - Johan den Dunnen
?/. 1 c.-515_-512del r.(?) p.(?) - VUS g.38211435_38211438del g.38352182_38352185del -512insTCTT - OTC_000257 - PubMed: Azevedo 2003 - rs57752938 Germline - 13/57 MboII - - Johan den Dunnen
?/. 1 c.-515_-512del r.(?) p.(?) - VUS g.38211435_38211438del g.38352182_38352185del -512insTCTT - OTC_000257 - PubMed: Azevedo 2003 - rs57752938 Germline - 7/57 MboII - - Johan den Dunnen
-/. 1 c.-515_-512del r.(?) p.(?) - benign g.38211435_38211438del g.38352182_38352185del -512insTCTT - OTC_000257 - PubMed: Azevedo 2003 - rs57752938 Germline - 13/103 MboII - - Johan den Dunnen
-/. 1 c.-515_-512del r.(?) p.(?) - benign g.38211435_38211438del g.38352182_38352185del -512insTCTT - OTC_000257 - PubMed: Azevedo 2003 - rs57752938 Germline - 18/36 MboII - - Johan den Dunnen
-/. 1 c.-515_-512del r.(?) p.(?) - benign g.38211435_38211438del g.38352182_38352185del -512insTCTT - OTC_000257 - PubMed: Azevedo 2003 - rs57752938 Germline - 21/57 MboII - - Johan den Dunnen
-/. 1 c.-515_-512del r.(?) p.(?) - benign g.38211435_38211438del g.38352182_38352185del -512insTCTT - OTC_000257 - PubMed: Azevedo 2003 - rs57752938 Germline - 38/103 MboII - - Johan den Dunnen
-/. 1 c.-515_-512del r.(?) p.(?) - benign g.38211435_38211438del g.38352182_38352185del -512insTCTT - OTC_000257 - PubMed: Azevedo 2003 - rs57752938 Germline - 7/36 MboII - - Johan den Dunnen
-/. 1 c.-515_-512del r.(?) p.(?) - benign g.38211435_38211438del g.38352182_38352185del -512insTCTT - OTC_000257 - PubMed: Azevedo 2003 - rs57752938 Germline - 3/57 MboII - - Johan den Dunnen
-/. 1 c.-515_-512del r.(?) p.(?) - benign g.38211435_38211438del g.38352182_38352185del -512insTCTT - OTC_000257 - PubMed: Azevedo 2003 - rs5917572 Germline - 3/103 MboII - - Johan den Dunnen
?/. 1 c.-441A>G r.(?) p.(?) - VUS g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 2/36 - - - Johan den Dunnen
?/. 1 c.-441A>G r.(?) p.(?) - VUS g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 16/103 - - - Johan den Dunnen
?/. 1 c.-441A>G r.(?) p.(?) - VUS g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 1/57 - - - Johan den Dunnen
?/. 1 c.-441A>G r.(?) p.(?) - VUS g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 13/57 - - - Johan den Dunnen
?/. 1 c.-441A>G r.(?) p.(?) - VUS g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 7/57 - - - Johan den Dunnen
-/. 1 c.-441A>G r.(?) p.(?) - benign g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 13/103 - - - Johan den Dunnen
-/. 1 c.-441A>G r.(?) p.(?) - benign g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 18/36 - - - Johan den Dunnen
-/. 1 c.-441A>G r.(?) p.(?) - benign g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 21/57 - - - Johan den Dunnen
-/. 1 c.-441A>G r.(?) p.(?) - benign g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 38/103 - - - Johan den Dunnen
-/. 1 c.-441A>G r.(?) p.(?) - benign g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 7/36 - - - Johan den Dunnen
-/. 1 c.-441A>G r.(?) p.(?) - benign g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 3/57 - - - Johan den Dunnen
-/. 1 c.-441A>G r.(?) p.(?) - benign g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 3/103 - - - Johan den Dunnen
-/. 1 c.-441A>G r.(?) p.(?) - benign g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 1/36 - - - Johan den Dunnen
-/. 1 c.-441A>G r.(?) p.(?) - benign g.38211509A>G g.38352256A>G (-441G>A) - OTC_000256 - PubMed: Azevedo 2003 - rs5917572 Germline - 1/57 - - - Johan den Dunnen
+/. 1 c.-366A>G r.(=) p.(=) - pathogenic g.38211584A>G g.38352331A>G - - OTC_000400 not in 300 control chromosomes - - - Germline - - - - - Lenka Dvořáková
?/. 1 c.-365G>A r.(?) p.(?) - VUS g.38211585G>A g.38352332G>A - - OTC_000255 - PubMed: Azevedo 2003 - rs5963030 Germline - 2/36 TseI;Fnu4HI;BbvI - - Johan den Dunnen
?/. 1 c.-365G>A r.(?) p.(?) - VUS g.38211585G>A g.38352332G>A - - OTC_000255 - PubMed: Azevedo 2003 - rs5963030 Germline - 16/103 TseI;Fnu4HI;BbvI - - Johan den Dunnen
?/. 1 c.-365G>A r.(?) p.(?) - VUS g.38211585G>A g.38352332G>A - - OTC_000255 - PubMed: Azevedo 2003 - rs5963030 Germline - 1/57 TseI;Fnu4HI;BbvI - - Johan den Dunnen
?/. 1 c.-365G>A r.(?) p.(?) - VUS g.38211585G>A g.38352332G>A - - OTC_000255 - PubMed: Azevedo 2003 - rs5963030 Germline - 7/57 TseI;Fnu4HI;BbvI - - Johan den Dunnen
-/. 1 c.-359A>G r.(?) p.(?) - benign g.38211591A>G g.38352338A>G - - OTC_000254 Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. PubMed: Azevedo 2003 - rs5917573 Germline - 24/103 - - - Johan den Dunnen
-/. 1 c.-359A>G r.(?) p.(?) - benign g.38211591A>G g.38352338A>G - - OTC_000254 Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. PubMed: Azevedo 2003 - rs5917573 Germline - 2/36 - - - Johan den Dunnen
-/. 1 c.-359A>G r.(?) p.(?) - benign g.38211591A>G g.38352338A>G - - OTC_000254 Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. PubMed: Azevedo 2003 - rs5917573 Germline - 6/57 - - - Johan den Dunnen
-/. 1 c.-359A>G r.(?) p.(?) - benign g.38211591A>G g.38352338A>G - - OTC_000254 Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. PubMed: Azevedo 2003 - rs5917573 Germline - 1/36 - - - Johan den Dunnen
-/. 1 c.-359A>G r.(?) p.(?) - benign g.38211591A>G g.38352338A>G - - OTC_000254 Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. PubMed: Azevedo 2003 - rs5917573 Germline - 1/57 - - - Johan den Dunnen
-/. 1 c.-359A>G r.(?) p.(?) - benign g.38211591A>G g.38352338A>G - - OTC_000254 Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. PubMed: Azevedo 2003 - rs5917573 Germline - 3/57 - - - Johan den Dunnen
-/. 1 c.-359A>G r.(?) p.(?) - benign g.38211591A>G g.38352338A>G - - OTC_000254 Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. PubMed: Azevedo 2003 - rs5917573 Germline - 4/103 - - - Johan den Dunnen
-/. 1 c.-359A>G r.(?) p.(?) - benign g.38211591A>G g.38352338A>G - - OTC_000254 Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. PubMed: Azevedo 2003 - rs5917573 Germline - 6/36 - - - Johan den Dunnen
-/. 1 c.-359A>G r.(?) p.(?) - benign g.38211591A>G g.38352338A>G - - OTC_000254 Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. PubMed: Azevedo 2003 - rs5917573 Germline - 2/57 - - - Johan den Dunnen
-/. 1 c.-359A>G r.(?) p.(?) - benign g.38211591A>G g.38352338A>G - - OTC_000254 Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. PubMed: Azevedo 2003 - rs5917573 Germline - 4/103 - - - Johan den Dunnen
?/. 1 c.-157T>G r.(?) p.(=) - VUS g.38211793T>G g.38352540T>G - - OTC_000450 - PubMed: Yang 2018 - - Germline - - - - - Johan den Dunnen
?/. 1 c.-157T>G r.(?) p.(=) - NA g.38211793T>G g.38352540T>G - - OTC_000450 in vitro expression analysis shows reduced reporter gene expression (0.75 in HuH-7 cells) PubMed: Yang 2018 - - In vitro (cloned) - - - - - Johan den Dunnen
?/. 1 c.-142G>A r.(?) p.(=) - VUS g.38211808G>A g.38352555G>A - - OTC_000451 no OTC activity PubMed: Yang 2018 - - Germline - - - - - Johan den Dunnen
+?/. 1 c.-142G>A r.(?) p.(=) - NA g.38211808G>A g.38352555G>A - - OTC_000451 in vitro expression analysis shows reduced reporter gene expression (0.4 in HuH-7 cells) PubMed: Yang 2018 - - In vitro (cloned) - - - - - Johan den Dunnen
?/. 1 c.-139A>G r.(?) p.(=) - VUS g.38211811A>G g.38352558A>G - - OTC_000452 - PubMed: Yang 2018 - - Germline - - - - - Johan den Dunnen
+?/. 1 c.-139A>G r.(?) p.(=) - NA g.38211811A>G g.38352558A>G - - OTC_000452 in vitro expression analysis shows reduced reporter gene expression (0.4 in HuH-7 cells) PubMed: Yang 2018 - - In vitro (cloned) - - - - - Johan den Dunnen
?/. 1 c.-116C>T r.(?) p.(=) - VUS g.38211834C>T g.38352581C>T - - OTC_000453 - PubMed: Yang 2018 - - Germline - - - - - Johan den Dunnen
+?/. 1 c.-116C>T r.(?) p.(=) - NA g.38211834C>T g.38352581C>T - - OTC_000453 in vitro expression analysis shows reduced reporter gene expression (0.8 in HuH-7 cells) PubMed: Yang 2018 - - In vitro (cloned) - - - - - Johan den Dunnen
?/. 1 c.-115C>T r.(?) p.(=) - VUS g.38211835C>T g.38352582C>T - - OTC_000454 - PubMed: Yang 2018 - - Germline - - - - - Johan den Dunnen
+?/. 1 c.-115C>T r.(?) p.(=) - NA g.38211835C>T g.38352582C>T - - OTC_000454 in vitro expression analysis shows reduced reporter gene expression (0.45 in HuH-7 cells); reduced binding HNF4 transcription factor PubMed: Yang 2018 - - In vitro (cloned) - - - - - Johan den Dunnen
+/. 1 c.-106C>A r.? p.? - pathogenic (recessive) g.38211844C>A g.38352591C>A - - OTC_000448 - PubMed: Kord 2026 - rs749748052 Germline yes - - - - Johan den Dunnen
+/. - c.-106C>A r.? p.? - pathogenic (recessive) g.38211844C>A g.38352591C>A - - OTC_000448 carries 11p14.3 deleton (VUS) PubMed: Kord 2026 - rs749748052 Germline yes - - - - Johan den Dunnen
+/. 1 c.-106C>A r.? p.? - pathogenic (recessive) g.38211844C>A g.38352591C>A - - OTC_000448 - PubMed: Kord 2026 - rs749748052 Germline/De novo (untested) yes - - - - Johan den Dunnen
+/. 1 c.-106C>A r.(-106C>A) p.(=) - pathogenic (recessive) g.38211844C>A g.38352591C>A - - OTC_000448 false positive MLPA analysis (0.25 exon 1 signal) PubMed: Hertzog 2022 - rs749748052 Germline - - - - - Johan den Dunnen
+/. 1 c.-106C>A r.(-106C>A) p.(=) - pathogenic (recessive) g.38211844C>A g.38352591C>A - - OTC_000448 - PubMed: Hertzog 2022 - rs749748052 Germline - - - - - Johan den Dunnen
+/. 1 c.-106C>A r.(-106C>A) p.(=) - pathogenic (recessive) g.38211844C>A g.38352591C>A - - OTC_000448 - PubMed: Hertzog 2022 - rs749748052 Germline - - - - - Johan den Dunnen
+/. 1 c.-106C>A r.(-106C>A) p.(=) - pathogenic (recessive) g.38211844C>A g.38352591C>A - - OTC_000448 >5x reduced in vitro expression luciferase reporter assay PubMed: Han 2022 - rs749748052 Germline - - - - - Johan den Dunnen
+/. 1 c.-106C>A r.(-106C>A) p.(=) - pathogenic (recessive) g.38211844C>A g.38352591C>A - - OTC_000448 >5x reduced in vitro expression luciferase reporter assay PubMed: Han 2022 - rs749748052 Germline - - - - - Johan den Dunnen
+?/. 1 c.-106C>A r.(-106C>A) p.(=) - likely pathogenic (recessive) g.38211844C>A g.38352591C>A - - OTC_000448 0.1 OTC activity PubMed: Yang 2018 - rs749748052 Germline - - - - - Johan den Dunnen
+?/. 1 c.-106C>A r.(-106C>A) p.(=) - likely pathogenic (recessive) g.38211844C>A g.38352591C>A - - OTC_000448 0.1 OTC activity PubMed: Yang 2018 - rs749748052 Germline - - - - - Johan den Dunnen
+?/. 1 c.-106C>A r.(-106C>A) p.(=) - likely pathogenic (recessive) g.38211844C>A g.38352591C>A - - OTC_000448 decreased OTC activity. PubMed: Yang 2018 - rs749748052 Germline - - - - - Johan den Dunnen
+?/. 1 c.-106C>A r.(?) p.(=) - NA g.38211844C>A g.38352591C>A - - OTC_000448 in vitro expression analysis shows reduced reporter gene expression (0.6 in HuH-7 cells); reduced binding HNF4 transcription factor PubMed: Yang 2018 - rs749748052 In vitro (cloned) - - - - - Johan den Dunnen
+?/. 1 c.-106C>A r.(?) p.(=) - NA g.38211844C>A g.38352591C>A - - OTC_000448 in vitro expression analysis shows reduced reporter gene expression (0.6 in HuH-7 cells); reduced binding HNF4 transcription factor PubMed: Yang 2018 - rs749748052 In vitro (cloned) - - - - - Johan den Dunnen
+?/. 1 c.-106C>A r.(?) p.(=) - NA g.38211844C>A g.38352591C>A - - OTC_000448 in vitro expression analysis shows reduced reporter gene expression (0.6 in HuH-7 cells); reduced binding HNF4 transcription factor PubMed: Yang 2018 - rs749748052 In vitro (cloned) - - - - - Johan den Dunnen
+/. 1 c.-106C>A r.(=|red) p.(=|red) - pathogenic (recessive) g.38211844C>A g.38352591C>A - - OTC_000448 - PubMed: Knerr 2022 - rs749748052 Germline yes - - - - Johan den Dunnen
+/. _1_3i c.(?_-1)_(298+1_299-1)del r.0? p.0? - pathogenic g.(?_38211949)_(38229131_38240594)del - - - OTC_000098 deletion exons 1-3 PubMed: Tuchman 1997 - - Germline - - - - - Bert Bakker
+/. _1_8i c.(?_-1)_(867+1_868-1)del r.0 p.0 - pathogenic g.(?_38211949)_(38268279_38271114)del - - - OTC_000357 - PubMed: Yamaguchi 2006 - - Germline - - - - - Bert Bakker
+/. _1_9i c.(?_-1)_(1005+1_1006-1)del r.0 p.0 - pathogenic g.(?_38211949)_(38271253_38280275)del - - - OTC_000355 - PubMed: Yamaguchi 2006 - - Germline - - - - - Bert Bakker
?/. _1_10_ c.(?_-1)_(*1_?)del r.0 p.0 - VUS g.(?_38211949)_(38280336_?)del - - - OTC_000210 deletion entire OTC gene PubMed: Suess 1992 - - Germline - - - - - Bert Bakker
?/. _1_10_ c.(?_-1)_(*1_?)del r.0 p.0 - VUS g.(?_38211949)_(38280336_?)del - - - OTC_000210 - PubMed: Yamaguchi 2006 - - Germline - - - - - Bert Bakker
?/. _1_10_ c.(?_-1)_(*1_?)del r.0 p.0 - VUS g.(?_38211949)_(38280336_?)del - - - OTC_000210 deletion entire OTC gene PubMed: Tuchman 2002 - - Germline - - - - - Bert Bakker
?/. 1 c.1A>G r.(?) p.0? - VUS g.38211950A>G g.38352697A>G - - OTC_000099 - PubMed: Oppliger Leibundgut 1997 - - Germline - - MboII;SfaNI - - Bert Bakker
?/. 1 c.1A>T r.(?) p.(Met1Leu) - VUS g.38211950A>T g.38352697A>T - - OTC_000361 - PubMed: Yamaguchi 2006 - - Germline - - - - - Bert Bakker
?/. 1 c.2T>C r.(?) p.(Met1Thr) - VUS g.38211951T>C g.38352698T>C - - OTC_000365 - PubMed: Yamaguchi 2006 - - Germline - - - - - Bert Bakker
?/. 1 c.3G>A r.(?) p.0? - VUS g.38211952G>A g.38352699G>A - - OTC_000211 - PubMed: Climent and Rubio 2002 - - Germline - - SfaNI;HpyCH4III - - Bert Bakker
?/. 1 c.26T>G r.(?) p.(Leu9X) - VUS g.38211975T>G g.38352722T>G c.25T>G - OTC_000264 not in 100 control chromosomes; OTC activity 0, Serum ammonia 430, Urine orotic acid 532, Serum citrulline 33.5, PubMed: Kim 2006 - - Germline - - - - - Bert Bakker
?/. 1 c.29dup r.(?) p.(Asn10LysfsTer6) - VUS g.38211978dup g.38352725dup - - OTC_000375 - PubMed: Yamaguchi 2006 - - Germline - - - - - Bert Bakker
?/. 1 c.29_32del r.(?) p.(Asn10MetfsTer27) - VUS g.38211978_38211981del g.38352725_38352728del - - OTC_000370 - PubMed: Yamaguchi 2006 - - Germline - - - - - Bert Bakker
?/. 1 c.34G>A r.(?) p.(Ala12Thr) - VUS g.38211983G>A g.38352730G>A - - OTC_000260 - Tanaka 2005 - - Germline - - TseI;CviRI;Fnu4HI;BbvI - - Bert Bakker
?/. 1 c.42del r.(?) p.(Phe14LeufsTer24) - VUS g.38211991del g.38352738del - - OTC_000379 Leader sequence; OTC activity 23% Hwu 2003 - - Germline - - - - - Bert Bakker
-/. 1 c.46A>G r.(?) p.(Asn16Asp ) - benign g.38211995A>G g.38352742A>G - - OTC_000390 100 individuals tested PubMed: Mitchell 2009 - - Germline - - - - - Johan den Dunnen
-/. 1 c.46A>G r.(?) p.(Asn16Asp) - benign g.38211995A>G g.38352742A>G - - OTC_000390 - PubMed: Mitchell 2009 - - Germline - - - - - Johan den Dunnen
?/. 1 c.53del r.(?) p.(His18ProfsTer20) - VUS g.38212002del g.38352749del - - OTC_000212 - PubMed: Tuchman 2002 - - Germline - - AvaII - - Bert Bakker
?/. 1 c.67C>T r.(?) p.(Arg23X) - VUS g.38212016C>T g.38352763C>T - - OTC_000011 Leader sequence Grompe 1991 - - Germline - - TaqI;AsuII - - Bert Bakker
+/. - c.67C>T r.(?) p.(Arg23Ter) - pathogenic g.38212016C>T g.38352763C>T - - OTC_000011 - PubMed: Martin-Hernandez 2014 - - Germline - - - - - Johan den Dunnen
-?/. - c.76C>T r.(?) p.(Arg26Trp) - likely benign g.38212025C>T g.38352772C>T OTC(NM_000531.5):c.76C>T (p.R26W, p.(Arg26Trp)) - OTC_000413 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.76C>T r.(?) p.(Arg26Trp) - likely benign g.38212025C>T - OTC(NM_000531.5):c.76C>T (p.R26W, p.(Arg26Trp)) - OTC_000413 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 c.77G>A r.(spl?) p.(Arg26Gln) - VUS g.38212026G>A g.38352773G>A - - OTC_000003 OTC activity 0% Grompe 1989 - - Germline - - - - - Bert Bakker
?/. 1 c.77G>A r.(spl?) p.(Arg26Gln) - VUS g.38212026G>A g.38352773G>A - - OTC_000003 OTC activity ?, Serum ammonia 1260, Urine orotic acid 3685, Serum citrulline 0, PubMed: Kim 2006 - - Germline - - - - - Bert Bakker
+/. - c.77G>A r.(?) p.(Arg26Gln) - pathogenic g.38212026G>A g.38352773G>A - - OTC_000003 1 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs68031618 Germline - 1/2795 individuals - - - Mohammed Faruq
?/. 1 c.77G>C r.(spl?) p.(Arg26Pro) - VUS g.38212026G>C g.38352773G>C - - OTC_000270 Leader sequence, CpG dinucleotide, possible donor splice site error PubMed: Yamaguchi 2006 - - Germline - - - - - Bert Bakker
?/. 1 c.77G>C r.(spl?) p.(Arg26Pro) - VUS g.38212026G>C g.38352773G>C - - OTC_000270 not in 100 control chromosomes; OTC activity 0, Serum ammonia 280, Urine orotic acid 783, Serum citrulline 11.2 PubMed: Kim 2006 - - Germline - - - - - Bert Bakker
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