All variants in the DHDDS gene

This database is one of the "Eye disease" gene variant databases.
Information The variants shown are described using the transcript reference sequence.

114 entries on 2 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     
?/. - c.-53T>G r.(?) p.(=) - VUS g.26759384T>G - DHDDS(NM_205861.3):c.-53T>G - DHDDS_000034 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. - c.63+14C>T r.(=) p.(=) - benign g.26759513C>T g.26433022C>T DHDDS(NM_024887.4):c.63+14C>T - DHDDS_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. - c.64-366A>T r.(=) p.(=) - likely benign g.26764293A>T - DHDDS(NM_205861.3):c.64-366A>T - DHDDS_000037 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.64-7T>C r.(=) p.(=) - VUS g.26764652T>C g.26438161T>C - - DHDDS_000025 - PubMed: Xu 2014 - - Germline - 1/314 case chromosomes - - - LOVD
+/. - c.109C>T r.(?) p.(Arg37Cys) - pathogenic g.26764704C>T g.26438213C>T - - DHDDS_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. - c.110G>A r.(?) p.(Arg37His) - pathogenic g.26764705G>A - - - DHDDS_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. - c.110G>A r.(?) p.(Arg37His) - pathogenic (dominant) g.26764705G>A - NM_205861:c.G110A (R37H) - DHDDS_000023 - PubMed: Hamdan 2017 - - De novo - - - - - Johan den Dunnen
+?/. - c.110G>A r.(?) p.(Arg37His) - likely pathogenic g.26764705G>A g.26438214G>A - - DHDDS_000023 - PubMed: Hamdan 2017 - - De novo - - - - - Johan den Dunnen
+/. 3 c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G g.26438228A>G - - DHDDS_000005 - PubMed: Sharon 2015, PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Dror Sharon
+/. 3 c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G g.26438228A>G - - DHDDS_000005 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Dror Sharon
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G g.26438228A>G - - DHDDS_000005 - PubMed: Venturini 2015 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G g.26438228A>G - - DHDDS_000005 - PubMed: Venturini 2015 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G g.26438228A>G - - DHDDS_000005 - PubMed: Venturini 2015 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G g.26438228A>G - - DHDDS_000005 - PubMed: Venturini 2015 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G g.26438228A>G - - DHDDS_000005 - PubMed: Venturini 2015 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Johan den Dunnen
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic (recessive) g.26764719A>G - - - DHDDS_000005 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Johan den Dunnen
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G - - DHDDS_000005 - PubMed: Stone 2017 - - Germline - - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G - - DHDDS_000005 - PubMed: Stone 2017 - - Germline - - - - - LOVD
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic g.26764719A>G g.26438228A>G - - DHDDS_000005 - PubMed: Biswas 2017 - rs147394623 Germline - - - - - LOVD
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic g.26764719A>G g.26438228A>G - - DHDDS_000005 - PubMed: Biswas 2017 - rs147394623 Germline - - - - - LOVD
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic g.26764719A>G g.26438228A>G - - DHDDS_000005 - PubMed: Biswas 2017 - rs147394623 Germline - - - - - LOVD
?/. - c.124A>G r.(?) p.(Lys42Glu) - VUS g.26764719A>G g.26438228A>G - - DHDDS_000005 - PubMed: Wang 2014 - rs147394623 Germline - - - - - LOVD
?/. - c.124A>G r.(?) p.(Lys42Glu) - VUS g.26764719A>G - DHDDS(NM_024887.3):c.124A>G (p.K42E) - DHDDS_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G p.Lys42Glu:c.124A/G; intronic variant, c.441-24A/G - DHDDS_000005 - PubMed: Hariri 2018 - - Germline ? - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G p.Lys42Glu:c.124A/G (homozygous) - DHDDS_000005 - PubMed: Hariri 2018 - - Germline ? - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G p.Lys42Glu:c.124A/G (homozygous) - DHDDS_000005 - PubMed: Hariri 2018 - - Germline ? - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G p.Lys42Glu:c.124A/G (homozygous) - DHDDS_000005 - PubMed: Hariri 2018 - - Germline ? - - - - LOVD
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G, p.Lys42Glu - DHDDS_000005 AJ founder mutation, heterozygous PubMed: Zampaglione 2020 - - Unknown ? - - - - LOVD
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G, p.Lys42Glu - DHDDS_000005 AJ founder mutation, heterozygous PubMed: Zampaglione 2020 - - Unknown ? - - - - LOVD
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic g.26764719A>G g.26438228A>G c.124A>G, p.Lys42Glu - DHDDS_000005 AJ founder mutation, homozygous PubMed: Zampaglione 2020 - - Unknown ? - - - - LOVD
+/. - c.124A>G r.(?) p.(Lys42Glu) - pathogenic g.26764719A>G g.26438228A>G c.124A>G, p.Lys42Glu - DHDDS_000005 AJ founder mutation, homozygous PubMed: Zampaglione 2020 - - Unknown ? - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G, p.K42E - DHDDS_000005 homozygous PubMed: Jauregui 2020 - - Unknown ? - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G, p.K42E - DHDDS_000005 homozygous PubMed: Jauregui 2020 - - Unknown ? - - - - LOVD
?/. 3 c.124A>G r.(?) p.(Lys42Glu) - VUS g.26764719A>G - p.K42E/p.K42E - DHDDS_000005 - PubMed: Matsui 2015 - - Germline - - - - - LOVD
+/. - c.124A>G r.(?) p.(Lys42Glu) ACMG pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G, p.(Lys42Glu) - DHDDS_000005 homozygous PubMed: Dineiro 2020 - - Germline ? - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zelinger 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zuchner 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zuchner 2011 - - Germline yes - - - - LOVD
+?/. - c.124A>G r.(?) p.(Lys42Glu) - likely pathogenic g.26764719A>G g.26438228A>G DHDDS c.124A>G (p.Lys42Glu) - DHDDS_000005 homozygous PubMed: Zuchner 2011 - - Germline yes - - - - LOVD
-?/. - c.140G>A r.(?) p.(Arg47Gln) - likely benign g.26764735G>A g.26438244G>A DHDDS(NM_024887.4):c.140G>A (p.R47Q) - DHDDS_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. - c.170A>G r.(?) p.(Lys57Arg) - VUS g.26764765A>G g.26438274A>G - - DHDDS_000007 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - - Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
-?/. - c.180+15C>T r.(=) p.(=) - likely benign g.26764790C>T - DHDDS(NM_001319959.2):c.-123+15C>T - DHDDS_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. - c.187C>T r.(?) p.(Arg63Trp) - VUS g.26769228C>T - DHDDS(NM_205861.3):c.187C>T (p.(Arg63Trp)) - DHDDS_000035 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 4 c.192G>A r.(?) p.(Trp64Ter) - likely pathogenic g.26769233G>A g.26442742G>A DHDDS c.192G > A (p.W64X) - DHDDS_000029 heterozygous PubMed: Sabry 2016 - - Germline yes - - - - LOVD
-?/. - c.220A>C r.(?) p.(Thr74Pro) - likely benign g.26769261A>C g.26442770A>C - - DHDDS_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. - c.283G>A r.(?) p.(Asp95Asn) ACMG VUS g.26769324G>A - - - DHDDS_000024 ACMG PM2, PP3, PP4; The patient's electroclinical phenotype shares some features with previous reports for this gene, namely ataxia and myoclonus. However onset is considerably later, and there is no history of developmental delay. Whilst functional studies support a damaging effect for this novel variant, but with a maternal DNA sample to confirm de novo status, we remain cautious and report this finding with moderate confidence. PubMed: Courage 2021, Journal: Courage 2021 - - De novo - - - - - Carolina Courage
+?/. 4 c.292C>T r.(?) p.(Arg98Trp) - likely pathogenic (recessive) g.26769333C>T g.26442842C>T - - DHDDS_000006 - PubMed: Kimchi 2018, PubMed: Sharon 2019 - - Germline - - - - - Dror Sharon
?/. - c.292C>T r.(?) p.(Arg98Trp) - VUS g.26769333C>T g.26442842C>T - - DHDDS_000006 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - rs754564043 Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
?/. - c.305G>A r.(?) p.(Ser102Asn) - VUS g.26769346G>A g.26442855G>A - - DHDDS_000026 - PubMed: Wang 2014 - - Germline - - - - - LOVD
-?/. - c.308G>A r.(?) p.(Arg103His) - likely benign g.26769349G>A - DHDDS(NM_205861.3):c.308G>A (p.(Arg103His)) - DHDDS_000036 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. - c.324-10C>T r.(=) p.(=) - benign g.26772797C>T g.26446306C>T DHDDS(NM_001319959.1):c.45-10C>T, DHDDS(NM_024887.4):c.324-10C>T - DHDDS_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. - c.324-10C>T r.(=) p.(=) - likely benign g.26772797C>T - DHDDS(NM_001319959.1):c.45-10C>T, DHDDS(NM_024887.4):c.324-10C>T - DHDDS_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.367G>A r.(?) p.(Asp123Asn) - VUS g.26772850G>A g.26446359G>A - - DHDDS_000008 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - rs140565871 Germline - 5/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
+?/. - c.441-24A>G r.spl? p.(?) - likely pathogenic g.26774026A>G g.26447535A>G p.Lys42Glu:c.124A/G; intronic variant, c.441-24A/G - DHDDS_000028 - PubMed: Hariri 2018 - - Germline ? - - - - LOVD
+?/. 5i c.441-24A>G r.440_543del102ins63 p.(Cys148Glufs*11) - likely pathogenic g.26774026A>G g.26447535A>G DHDDS c.441-24A > G, c.440_543del102ins63 (p.C148EfsX11) - DHDDS_000028 creates a cryptic donor splice site (with score of 0.99 rather than 0.65 for the normal exon 6 donor site) leading to loss of exon 6, a 63 base insertion into intron 5 - tested on mRNA level; heterozygous PubMed: Sabry 2016 - - Germline yes - - - - LOVD
?/. - c.514G>C r.(?) p.(Gly172Arg) - VUS g.26774123G>C - DHDDS(NM_001319959.1):c.235G>C (p.G79R) - DHDDS_000031 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.516G>C r.(?) p.(Gly172=) - likely benign g.26774125G>C g.26447634G>C DHDDS(NM_001319959.1):c.237G>C (p.G79=) - DHDDS_000020 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. - c.542+8G>A r.(=) p.(=) - benign g.26774159G>A g.26447668G>A DHDDS(NM_024887.4):c.542+8G>A - DHDDS_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. - c.552T>A r.(?) p.(Ser184=) - likely benign g.26784291T>A - DHDDS(NM_001319959.1):c.273T>A (p.S91=) - DHDDS_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. - c.614G>A r.(?) p.(Arg205Gln) - likely pathogenic g.26784353G>A g.26457862G>A DHDDS(NM_024887.4):c.614G>A (p.R205Q) - DHDDS_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/. - c.614G>A r.(?) p.(Arg205Gln) ACMG pathogenic g.26784353G>A - - - DHDDS_000017 ACMG PS3, PM1, PM2, PP2, PP3, PP5; Although there is no history of developmental delay, the patient's electroclinical phenotype shares several other features with previous reports for this gene, including early onset ataxia and subsequent myoclonus. Functional studies support the damaging in silico predications for this novel variant, but without parental DNA samples to confirm de novo status, we remain cautious and report this finding with moderate confidence. PubMed: Courage 2021, Journal: Courage 2021 - - De novo - - - - - Carolina Courage
?/. - c.614G>A r.(?) p.(Arg205Gln) ACMG VUS (!) g.26784353G>A - - - DHDDS_000017 ACMG: PM6, PM2_SUP, PP3: class 3 - ClinVar-000570739 - Germline ? - - - - Andreas Laner
+/. - c.616A>G r.(?) p.(Thr206Ala) - pathogenic (recessive) g.26784355A>G - - - DHDDS_000022 - PubMed: Kimchi 2018 - - Germline - - - - - Johan den Dunnen
+/. - c.616A>G r.(?) p.(Thr206Ala) - pathogenic g.26784355A>G g.26457864A>G - - DHDDS_000022 - PubMed: Biswas 2017 - - Germline - - - - - LOVD
+/. - c.632G>A r.(?) p.(Arg211Gln) - pathogenic g.26784371G>A g.26457880G>A DHDDS(NM_024887.4):c.632G>A (p.R211Q) - DHDDS_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. - c.632G>A r.(?) p.(Arg211Gln) ACMG pathogenic g.26784371G>A - - - DHDDS_000018 ACMG PS1, PS2, PS3, PM1, PM2, PP2, PP3, PP5; The patient's electroclinical phenotype is similar to previous reports for this gene, notably onset of myoclonus and ataxia in the first decade of life on a background of global developmental delay. The confirmed de novo has been reported previously as pathogenic in a patient with developmental and epileptic encephalopathy and our functional studies support the damaging in silico predications. It is therefore with high confidence that we expand the DHDDS clinical spectrum to PME. PubMed: Courage 2021, Journal: Courage 2021 - - De novo - - - - - Carolina Courage
+/. - c.632G>A r.(?) p.(Arg211Gln) - pathogenic g.26784371G>A - DHDDS(NM_024887.4):c.632G>A (p.R211Q) - DHDDS_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+?/. - c.632G>A r.(?) p.(Arg211Gln) - likely pathogenic (dominant) g.26784371G>A g.26457880G>A - - DHDDS_000018 - PubMed: Hamdan 2017 - - De novo - - - - - Johan den Dunnen
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