Unique variants in the TMEM216 gene

Information The variants shown are described using the NM_001173990.2 transcript reference sequence.

23 entries on 1 page. Showing entries 1 - 23.
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Effect     

Reported     

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     
+/., ?/. 2 - c.*558G>A r.(?) p.? - pathogenic, VUS g.61166306G>A - c.*558G >A, TMEM216: c.*558G>A - TMEM216_000020 - PubMed: M'hamdi 2014, PubMed: M'hamdi-2014 - - Germline, Unknown - - - - - LOVD
+/. 2 - c.-272_228{0} r.0? p.0? - pathogenic (recessive) g.61150363_61161447del g.61382891_61393975del c.-9741_228del, hg38 g.61,382,891-61,393,975del - TMEM216_000027 - PubMed: Malka 2024 - - Germline - - - - - Johan den Dunnen
+/. 18 _1 c.-69G>A r.=|red p.=|red - NA, pathogenic (recessive) g.61160035G>A g.61392563G>A - - TMEM216_000025 luciferase reporter gene assay shows reduced promotor activity, variant has reduced promotor activity PubMed: Malka 2024 - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
+/. 46 _1 c.-69G>T r.=|red p.=|red - NA, pathogenic (recessive) g.61160035G>T g.61392563G>T - - TMEM216_000026 luciferase reporter gene assay showed reduced promotor activity, variant has reduced promotor activity PubMed: Malka 2024 - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
-?/. 1 - c.5T>C r.(?) p.(Leu2Pro) - likely benign g.61160108T>C g.61392636T>C TMEM216(NM_001173990.2):c.5T>C (p.(Leu2Pro)) - TMEM216_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. 2 - c.35-17C>T r.(=) p.(=) - benign g.61160686C>T g.61393214C>T TMEM216(NM_001173990.3):c.35-17C>T, TMEM216(NM_001173991.2):c.35-17C>T - TMEM216_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
+/. 2 - c.35-2A>G r.spl, r.spl? p.? - pathogenic, pathogenic (recessive) g.61160701A>G g.61393229A>G TMEM216(NM_001173991.2):c.35-2A>G - TMEM216_000012 VKGL data sharing initiative Nederland PubMed: Malka 2024 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Rotterdam
-?/. 2 - c.140T>C r.(?) p.(Val47Ala) - likely benign g.61161359T>C - TMEM216(NM_001173990.2):c.140T>C (p.(Val47Ala)), TMEM216(NM_001173990.3):c.140T>C (p.V47A) - TMEM216_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Utrecht
-?/., ?/. 2 - c.211G>T r.(?) p.(Val71Leu) - likely benign, VUS g.61161430G>T - TMEM216(NM_001173990.3):c.211G>T (p.V71L), TMEM216(NM_001330285.1):c.28G>T (p.V10L) - TMEM216_000024 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Utrecht
+?/. 1 - c.216T>C r.(=) p.(=) - likely pathogenic g.61161435T>C g.61393963T>C NM_001173990.2:c.216T>C - TMEM216_000019 - PubMed: Bachmann-Gagescu 2015 - - Germline - - - - - LOVD
+/. 3 - c.217C>T r.(?) p.(Arg73Cys) - pathogenic g.61161436C>T g.61393964C>T C217T, R73C, NM_001173990.2:c.217C>T - TMEM216_000003 - PubMed: Bachmann-Gagescu 2015, PubMed: Valente 2010 - - Germline, Unknown - - - - - LOVD
+/. 4 - c.218G>A r.(?) p.(Arg73His) - pathogenic g.61161437G>A g.61393965G>A G218A, R73H - TMEM216_000002 - PubMed: Valente 2010 - - Unknown - - - - - LOVD
+/., +?/. 85 - c.218G>T r.(?) p.(Arg73Leu) - likely pathogenic, pathogenic g.61161437G>T g.61393965G>T c.218G>T; p.R73L, c.35G>T, R12L, G218T, R73L, NM_001173990.2:c.218G>T - TMEM216_000001 different transcript: NM_001173991.2(TMEM216):c.218G>T, Founder Mutation, 1 more item PubMed: Bachmann-Gagescu 2015, PubMed: Brooks 2018, PubMed: Edvardson 2010, PubMed: Summers 2017, 1 more item - - Germline, Unknown ? - - - - LOVD
+/+?, +/. 13 - c.230G>C r.(?), r.229_230ins230-46_230-1 p.(Gly77Ala), p.? - pathogenic g.61165246G>C g.61397774G>C G230C G77Asplice: T78KfsX30 - TMEM216_000006, TMEM216_000008 - PubMed: Valente 2010 - - Germline, Unknown - 0/500 CON - - - Anne Polvi
-?/. 1 - c.253C>A r.(?) p.(Arg85=) - likely benign g.61165269C>A g.61397797C>A TMEM216(NM_001173991.2):c.253C>A (p.R85=) - TMEM216_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/+?, +/., +?/. 9 - c.253C>T r.(253c>u), r.(?) p.(Arg85*), p.(Arg85Ter) - likely pathogenic, pathogenic g.61165269C>T g.61397797C>T c.253C>T; p.R85*, C253T, R85X, NM_001173990.2:c.253C>T, 1 more item - TMEM216_000007 different transcript: NM_001173990.3(TMEM216):c.253C>T, VKGL data sharing initiative Nederland PubMed: Bachmann-Gagescu 2015, PubMed: Brooks 2018, PubMed: Summers 2017, PubMed: Valente 2010 - - CLASSIFICATION record, Germline, Unknown ? 0/496 CON - - - VKGL-NL_Leiden, Anne Polvi
?/. 1 - c.254G>A r.(?) p.(Arg85Gln) - VUS g.61165270G>A - TMEM216(NM_001330285.1):c.71G>A (p.R24Q) - CPSF7_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 2 - c.264G>A r.(?) p.(Pro88=) - benign g.61165280G>A g.61397808G>A TMEM216(NM_001173991.2):c.264G>A (p.P88=), TMEM216(NM_001173991.3):c.264G>A (p.P88=) - TMEM216_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen
?/. 1 - c.289T>A r.(?) p.(Phe97Ile) ACMG VUS g.61165305T>A g.61397833T>A TMEM216, c.289T>A, p.Phe97Ile, heterozygous - TMEM216_000023 different transcript: ENM_001173991.2(TMEM216):c.289T>A, p.Phe97Ile, compound heterozygous PubMed: Perea-Romero 2021 - - Germline yes - - - - LOVD
+/+?, +/. 7 - c.341T>G r.(341u>g), r.(?) p.(Leu114Arg) - pathogenic g.61165357T>G g.61397885T>G T341G, L114R - TMEM216_000005 Mutation leads to unstable protein product. PubMed: Valente 2010 - - Germline, Unknown - 0/766 CON - - - Anne Polvi
+/. 3 - c.398T>G r.(?) p.(Leu133*), p.(Leu133Ter) - pathogenic g.61165414T>G g.61397942T>G NM_001173990.2:c.398T>G, T398G, L133X - TMEM216_000004 - PubMed: Bachmann-Gagescu 2015, PubMed: Valente 2010 - - Germline, Unknown - - - - - LOVD
-/. 2 - c.432-11_432-10insA r.(=) p.(=) - benign g.61165731_61165732insA g.61398259_61398260insA 1 more item - TMEM216_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen
-/. 2 - c.432-1G>C r.spl? p.? - benign g.61165741G>C g.61398269G>C TMEM216(NM_001173990.3):c.432-1G>C, TMEM216(NM_001173991.2):c.440G>C (p.R147T) - TMEM216_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen
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