Unique variants in the POMGNT1 gene

This database is one of the gene variant databases from the Leiden Muscular Dystrophy pages
Information The variants shown are described using the transcript reference sequence.

233 entries on 3 pages. Showing entries 1 - 100.
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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     
?/. 1 - c.-409G>A r.(?) p.(=) - VUS g.46685735C>T g.46220063C>T LURAP1(NM_001013615.2):c.563C>T (p.(Ala188Val)) - LURAP1_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.-390T>C r.(?) p.(=) - likely benign g.46685716A>G g.46220044A>G LURAP1(NM_001013615.2):c.544A>G (p.(Arg182Gly)) - LURAP1_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.-51G>A r.(?) p.(=) - likely benign g.46685377C>T - POMGNT1(NM_001243766.2):c.-51G>A - LURAP1_000041 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/+?, +/. 2 1i_8i, 2i_8i c.(-51+1_-50-1)_(751+1_752-1)del r.? p.0?, p.? - pathogenic g.(46660074_46660224)_(46663544_46685376)del - deletion exons 2–8 - POMGNT1_000134 1 Italian MDDGA3 (MEB) patient (com-het) PubMed: Saredi 2012 - - Germline, SUMMARY record - - - - - Johan den Dunnen
-/. 2 2 c.-11G>A r.(?) p.(=) - benign g.46663504C>T g.46197832C>T POMGNT1(NM_001243766.2):c.-11G>A - POMGNT1_000040 other pathogenic change in one patient, VKGL data sharing initiative Nederland PubMed: Godfrey 2007 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_AMC
+?/. 1 1 c.0? r.0? p.0? - likely pathogenic g.45862062_46510920dup - POMGNT1 - MMACHC_000047 heterozygous, both parents wild type; t(2;14)(q37.3;q13) with duplication in chromosome 1p34.1 PubMed: Hanemaaijer 2009 - - De novo yes - - - - LOVD
+/. 2 17i_23_ c.1539+184_*38{0} r.? p.? - pathogenic, pathogenic (recessive) g.46652588_46657586del g.46186916_46191914del 1539+184_1983+2354del4972 - POMGNT1_000113 5.0 Kb deletion exon 17-22 PubMed: Vuillaumier-Barrot 2011 - - Germline, SUMMARY record - - - - - Johan den Dunnen, Anne Polvi
+/. 2 17i_22_, 17i_23_ c.1539+261_*38{0} r.? p.? - pathogenic, pathogenic (recessive) g.46652537_46657509del g.46186865_46191837del 1539+261_1983+2405del4972 - POMGNT1_000062 5.0 Kb deletion exon 17-22 PubMed: Vuillaumier-Barrot 2011 - - Germline, SUMMARY record - - - - - Johan den Dunnen, Anne Polvi
?/. 5 22 c.? r.(?) p.(Thr660Ile), p.? - VUS g.? - 1927T>G (F643V), 1933G>C (E645Q), 1979C>T (T660I) - NPHS2_000000, POMGNT1_000000 no second variant PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
+/. 1 - c.1A>T r.(?) p.(Met1?) - pathogenic g.46663493T>A g.46197821T>A - - POMGNT1_000193 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 2 c.16C>T r.(?) p.(Pro6Ser) - VUS g.46663478G>A g.46197806G>A - - POMGNT1_000241 no second variant PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
+/+?, +/. 2 2 c.25dup r.(25dup), r.(?) p.(Leu9Profs*20) - pathogenic g.46663473dup g.46197801dup 25_26insC - POMGNT1_000052 1 German MDDGA3 (MEB) patient (com-het) PubMed: Hehr 2007 - - Germline, SUMMARY record - - - - - Johan den Dunnen, Rosário dos Santos
?/. 2 2 c.38T>C r.(?) p.(Phe13Ser) - VUS g.46663456A>G g.46197784A>G POMGNT1(NM_001243766.1):c.38T>C (p.F13S) - POMGNT1_000183 no second variant, VKGL data sharing initiative Nederland PubMed: Nallamilli 2018 - - CLASSIFICATION record, Germline - - - - - Madhuri Hegde, VKGL-NL_Rotterdam
?/. 3 2 c.46C>T r.(?) p.(Arg16Trp) - VUS g.46663448G>A g.46197776G>A - - POMGNT1_000010 no second variant PubMed: Nallamilli 2018 - rs34058684 Germline - 0.01 - - - Johan den Dunnen, Madhuri Hegde
?/. 1 - c.56G>A r.(?) p.(Arg19Gln) - VUS g.46663438C>T g.46197766C>T 667G>A;56G>A - POMGNT1_000259 - PubMed: Song 2021 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.73T>G r.(?) p.(Trp25Gly) - VUS g.46663421A>C - POMGNT1(NM_017739.4):c.73T>G (p.(Trp25Gly)) - LURAP1_000036 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 2 c.107G>A r.(?) p.(Arg36Gln) - VUS g.46663387C>T g.46197715C>T - - POMGNT1_000240 no second variant PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
?/. 1 2i c.120+3A>G r.spl? p.? - VUS g.46663371T>C g.46197699T>C - - POMGNT1_000239 - PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
-?/. 4 - c.120+13C>T r.(=) p.(=) - likely benign g.46663361G>A g.46197689G>A 1 more item - POMGNT1_000182 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_AMC
-/. 1 - c.121-278A>G r.(=) p.(=) - benign g.46663034T>C g.46197362T>C POMGNT1(NM_001290129.2):c.-1A>G - LURAP1_000020 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. 1 - c.121-228A>G r.(=) p.(=) - likely benign g.46662984T>C - POMGNT1(NM_001290129.2):c.50A>G (p.(His17Arg)) - LURAP1_000040 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 3 c.172A>G r.(?) p.(Ile58Val) - VUS g.46662705T>C g.46197033T>C - - POMGNT1_000238 - PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
+/., +?/. 9 3, 4 c.187C>T r.(?) p.(Arg63*), p.(Arg63Ter) - likely pathogenic, pathogenic, pathogenic (recessive) g.46662690G>A g.46197018G>A 281C>T (R63X), c.187C>T, 1 more item - POMGNT1_000009, POMGNT1_000076 VKGL data sharing initiative Nederland PubMed: Mercuri 2009, PubMed: Panneman 2023, PubMed: Song 2021, PubMed: Taniguchi 2003, OMIM:var0009, 1 more item - - CLASSIFICATION record, Germline, Unknown yes 1/309 unresolved RP cases - - - Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Groningen, Daan Panneman
-/. 2 3i c.235+33T>G r.(?) p.(=) - benign g.46662609A>C g.46196937A>C - - POMGNT1_000041 found in several patients from website {DBsub-Emory}, PubMed: Godfrey 2007 - - Germline, Unknown - - - - - Johan den Dunnen, Madhuri Hegde
-/. 1 - c.236-16C>G r.(=) p.(=) - benign g.46662537G>C g.46196865G>C POMGNT1(NM_017739.3):c.236-16C>G - POMGNT1_000180 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/., ?/. 2 3i c.236-13T>C r.(=), r.(?) p.(=) - likely benign, VUS g.46662534A>G g.46196862A>G POMGNT1(NM_017739.3):c.236-13T>C - POMGNT1_000065 VKGL data sharing initiative Nederland from website {DBsub-Emory} - - CLASSIFICATION record, Unknown - - - - - Madhuri Hegde, VKGL-NL_Rotterdam
?/. 1 - c.244C>G r.(?) p.(Leu82Val) ACMG VUS g.46662513G>C g.46196841G>C POMGNT1 c.244C>G, p.(Leu82Val) - POMGNT1_000274 heterozygous, probably non-causal incidental finding PubMed: Zhu 2022 - - Germline/De novo (untested) ? - - - - LOVD
?/. 1 4 c.244C>T r.(?) p.(=) - VUS g.46662513G>A g.46196841G>A - - POMGNT1_000059 - - - rs34212808 Germline - 0.34 - - - Johan den Dunnen
?/. 1 - c.251G>A r.(?) p.(Arg84His) - VUS g.46662506C>T - POMGNT1(NM_001243766.1):c.251G>A (p.R84H) - LURAP1_000024 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.251G>T r.(?) p.(Arg84Leu) - VUS g.46662506C>A - POMGNT1(NM_001243766.1):c.251G>T (p.(Arg84Leu)) - LURAP1_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 4 c.266G>A r.(?) p.(Arg89Gln) - VUS g.46662491C>T g.46196819C>T - - POMGNT1_000237 - PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
?/. 1 4 c.268C>T r.(?) p.(Arg90Cys) - VUS g.46662489G>A g.46196817G>A - - POMGNT1_000236 no second variant PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
?/. 1 4 c.269G>A r.(?) p.(Arg90His) - VUS g.46662488C>T g.46196816C>T - - POMGNT1_000235 no second variant PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
?/. 1 - c.275G>T r.(?) p.(Gly92Val) - VUS g.46662482C>A - POMGNT1(NM_001243766.1):c.275G>T (p.G92V) - POMGNT1_000178 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 4 c.286C>T r.(?) p.(Arg96Trp) - VUS g.46662471G>A g.46196799G>A - - POMGNT1_000234 no second variant PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
+/. 3 - c.296T>C r.(?) p.(Leu99Pro) - pathogenic, pathogenic (recessive) g.46662461A>G g.46196789A>G - - POMGNT1_000156 - PubMed: Fu 2017, PubMed: Song 2021 - - Germline - - - - - Johan den Dunnen, Xiaona Fu
-/., -?/. 4 - c.301G>A r.(?) p.(Val101Ile) - benign, likely benign g.46662456C>T g.46196784C>T POMGNT1(NM_001243766.1):c.301G>A (p.V101I), POMGNT1(NM_001243766.2):c.301G>A (p.V101I) - POMGNT1_000192 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_Nijmegen, VKGL-NL_AMC
+/. 2 4 c.304G>T r.(?) p.(Glu102Ter) ACMG pathogenic (recessive) g.46662453C>A g.46196781C>A - - POMGNT1_000272 ACMG PVS1, PM2, PP3, PP4 PubMed: Kritioti 2021 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.333T>C r.(?) p.(Tyr111=) - likely benign g.46662424A>G - POMGNT1(NM_001243766.1):c.333T>C (p.Y111=) - LURAP1_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/+?, +/. 5 4 c.351del r.(351del), r.(?) p.(Thr118Argfs*26) - pathogenic, pathogenic (recessive) g.46662406del, g.46662407del g.46196735del 350delC, POMGNT1:c.351del - POMGNT1_000075, POMGNT1_000196 1 more item PubMed: Colombo-2020, PubMed: Mercuri 2009, PubMed: Saredi 2012 - rs386834028 Germline, SUMMARY record - - - - - Johan den Dunnen
-/. 2 4i c.355-27C>T r.(?) p.(=) - benign g.46661776G>A g.46196104G>A - - POMGNT1_000042 found in one patient from website {DBsub-Emory}, PubMed: Godfrey 2007 - - Germline, Unknown - - - - - Johan den Dunnen, Madhuri Hegde
?/. 1 5 c.358C>G r.(?) p.(Leu120Val) - VUS g.46661746G>C g.46196074G>C - - POMGNT1_000233 no second variant PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
+?/. 19 4 c.359T>G r.(?) p.(Leu120Arg) - likely pathogenic g.46661745A>C g.46196073A>C POMGNT1 c.359T>G, p.Leu120Arg, POMGNT1:NM_001290129:exon4:c.359T>G:p.L120R - POMGNT1_000256 homozygous PubMed: Chen 2020, PubMed: Wang 2016 - - Germline ?, yes - - - - LOVD
+?/. 3 5 c.385C>T r.(?) p.(Arg129Trp) ACMG likely pathogenic, likely pathogenic (dominant) g.46661719G>A g.46196047G>A POMGNT1 c.385C > T (p.Arg129Trp) - POMGNT1_000271 ACMG PM1, PM2, PM3, PP2, PP3, PP4, heterozygous PubMed: Borisovna 2019, PubMed: Kritioti 2021 - rs375431575 Germline yes - - - - Johan den Dunnen
+?/. 1 - c.386G>A r.(?) p.(Arg129Gln) ACMG likely pathogenic (recessive) g.46661718C>T - - - POMGNT1_000254 ACMG PM1, PM2, PP2, PP3, PP4 PubMed: Mohammadi 2021 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.387G>A r.(?) p.(Arg129=) - likely benign g.46661717C>T g.46196045C>T POMGNT1(NM_001243766.2):c.387G>A (p.R129=) - LURAP1_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 5 c.418A>G r.(?) p.(Thr140Ala) - VUS g.46661686T>C g.46196014T>C - - POMGNT1_000232 no second variant PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
?/. 1 - c.419C>T r.(?) p.(Thr140Met) - VUS g.46661685G>A g.46196013G>A POMGNT1(NM_017739.3):c.419C>T (p.T140M) - LURAP1_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 5i_6 c.421-29_452del r.spl p.? - pathogenic g.46661571_46661631del g.46195899_46195959del 421-29_452del61 - POMGNT1_000049 - PubMed: Bouchet 2007 - - Germline yes - - - - Johan den Dunnen
-?/., ?/. 2 5i c.421-7C>A r.(=), r.spl p.(=) - likely benign, VUS g.46661603G>T g.46195931G>T POMGNT1(NM_001243766.1):c.421-7C>A - POMGNT1_000231 VKGL data sharing initiative Nederland PubMed: Nallamilli 2018 - - CLASSIFICATION record, Germline - - - - - Madhuri Hegde, VKGL-NL_Rotterdam
+/. 1 5i_9i c.(420+1_421-1)_(879+1_880-1)del r.? p.? - pathogenic (recessive) g.(46659598_46659945)_(46661597_46661683)del g.(46193926_46194273)_(46195925_46196011)del del ex6-9 - POMGNT1_000262 enzyme activity, protein expression and subcellular localization assessed in HeLa cells PubMed: Song 2021 - - Germline - - - - - Johan den Dunnen
+?/. 1 - c.445del r.(?) p.(Phe149Leufs*19) - likely pathogenic g.46661572del g.46195900del POMGNT1 Phe149 frameshift 167 stop - POMGNT1_000015 probably c.447del (reference Taniguchi et al., 2007); heterozygous PubMed: Voglmeir 2011 - - Unknown ? - - - - LOVD
+/+?, +/. 2 6 c.447del r.(447del), r.(?) p.(Phe149Leufs*19) - pathogenic g.46661572del g.46195900del 447delT, 541delT - POMGNT1_000015 1 Japan MDDGA3 (MEB) patient (com-het) PubMed: Taniguchi 2003, PubMed: Manya 2003 - - Germline, SUMMARY record - - - - - Johan den Dunnen
+/. 2 6 c.447dup r.(?) p.(Asp150*) - pathogenic g.46661572dup g.46195900dup 447dupT, 447dupT A(sp150fsX) - POMGNT1_000028 not in 188 control chromosomes PubMed: Clement, PubMed: Longman 2004 - - Germline - - - - - Johan den Dunnen
?/. 1 6 c.452C>T r.(?) p.(Thr151Met) - VUS g.46661565G>A g.46195893G>A - - POMGNT1_000230 - PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
-/. 1 6 c.458C>G r.(?) p.(Ser153*) - benign g.46661559G>C g.46195887G>C - - POMGNT1_000046 - PubMed: Manya 2008 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.461C>A r.(?) p.(Pro154His) - VUS g.46661556G>T g.46195884G>T - - LURAP1_000019 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 6 c.461C>T r.(?) p.(Pro154Leu) - VUS g.46661556G>A g.46195884G>A - - POMGNT1_000229 no second variant PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
+/. 1 - c.466G>A r.(?) p.(Glu156Lys) - pathogenic (recessive) g.46661551C>T g.46195879C>T - - POMGNT1_000251 1 more item PubMed: Xu 2016, PubMed: Xu 2016 - - Germline - - - - - Johan den Dunnen
+/. 1 6 c.477dup r.(?) p.(Met160Hisfs*21) - pathogenic g.46661541dup g.46195869dup 477dupC - POMGNT1_000228 no second variant PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
?/. 1 - c.499G>A r.(?) p.(Val167Ile) - VUS g.46661518C>T g.46195846C>T - - POMGNT1_000249 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - - Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
?/. 1 6 c.503C>T r.(?) p.(Ala168Val) - VUS g.46661514G>A g.46195842G>A - - POMGNT1_000227 - PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
+/. 1 - c.511C>T r.(?) p.(Arg171Ter) - pathogenic (recessive) g.46661506G>A g.46195834G>A - - POMGNT1_000270 - PubMed: Song 2021 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.512G>T r.(?) p.(Arg171Leu) - VUS g.46661505C>A g.46195833C>A - - POMGNT1_000191 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/., +?/+? 2 6 c.526A>C r.(526a>c), r.(?) p.(Thr176Pro) - likely pathogenic, pathogenic g.46661491T>G g.46195819T>G Thr176Pro - POMGNT1_000043 1 MDDGA3 (MEB) patient (hom) with unknown ethnicity PubMed: Godfrey 2007 - - Germline, SUMMARY record - - - - - Johan den Dunnen
+/. 1 - c.528dup r.(?) p.(Val177CysfsTer4) - pathogenic (recessive) g.46661489dup g.46195817dup c.528dupT - POMGNT1_000269 - PubMed: Song 2021 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.534+15C>T r.(=) p.(=) - likely benign g.46661468G>A - POMGNT1(NM_017739.3):c.534+15C>T - LURAP1_000029 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. 1 - c.550C>T r.(?) p.(His184Tyr) - VUS g.46660618G>A g.46194946G>A - - LURAP1_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 7 c.561C>G r.(?) p.(Asp187Glu) - VUS g.46660607G>C g.46194935G>C - - POMGNT1_000226 - PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
+/., +?/+? 3 7 c.575T>C r.(575u>c), r.(?) p.(Leu192Pro) - likely pathogenic, pathogenic, pathogenic (recessive) g.46660593A>G g.46194921A>G - - POMGNT1_000150 1 Chinese MDDGA3 (MEB) patient (com-het) PubMed: Jiao 2013, PubMed: Song 2021 - - Germline, SUMMARY record - - - - - Johan den Dunnen
+/+?, +/. 3 7 c.593del r.(593del), r.(?) p.(Ser198Thrfs*43) - pathogenic g.46660575del g.46194903del 593del premTerm240, 593delG - POMGNT1_000051 1 American and 1 German MDDGA3 (MEB) patient (both com-het) PubMed: Hehr 2007 - - Germline, SUMMARY record - - - - - Johan den Dunnen, Rosário dos Santos
+/., +?/. 3 7 c.594C>G r.(?) p.(Ser198Arg) - likely pathogenic, likely pathogenic (recessive), pathogenic (recessive) g.46660574G>C g.46194902G>C POMGNT1 S198R - POMGNT1_000137 protein concentrations of the expressed mutant rPOMGnT1 protein diminished; heterozygous PubMed: Biancheri 2006, PubMed: Mercuri 2009, PubMed: Voglmeir 2011 - - Germline, SUMMARY record, Unknown -, ? - - - - Johan den Dunnen, Anne Polvi
+?/. 1 7 c.617G>A r.(?) p.(Trp206Ter) - likely pathogenic g.46660551C>T g.46194879C>T - - POMGNT1_000255 - PubMed: Ganapathy 2019 - - Germline - - - - - Johan den Dunnen
+?/. 1 - c.622G>A r.(?) p.(Asp208Asn) - likely pathogenic g.46660546C>T g.46194874C>T - - POMGNT1_000253 - PubMed: Stone 2017 - - Germline - - - - - LOVD
+/., +?/. 3 7 c.630G>T r.(?) p.(Trp210Cys) - likely pathogenic, pathogenic g.46660538C>A g.46194866C>A POMGNT1(NM_001243766.1):c.630G>T (p.W210C), POMGNT1(NM_001243766.2):c.630G>T (p.W210C) - POMGNT1_000050 not in 200 control chromosomes, VKGL data sharing initiative Nederland PubMed: Bouchet 2007 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Groningen
+/., +?/. 7 7 c.636C>T r.(=), r.(?), r.535_652del, r.spl p.(=), p.(Phe212=), p.Asp179Valfs*23 - likely pathogenic, likely pathogenic (recessive), pathogenic g.46660532G>A g.46194860G>A POMGNT1(NM_001243766.2):c.636C>T (p.F212=) - POMGNT1_000030 strong NMD for variant in muscle, VKGL data sharing initiative Nederland from website {DBsub-Emory}, PubMed: Bouchet 2007, PubMed: Cummings 2017, PubMed: Mercuri 2009, 1 more item - - CLASSIFICATION record, Germline, SUMMARY record, Unknown - - - - - Johan den Dunnen, Anne Polvi, Madhuri Hegde, VKGL-NL_Groningen
+/. 1 - c.637G>A r.(?) p.(Val213Met) - pathogenic (recessive) g.46660531C>T g.46194859C>T - - POMGNT1_000268 - PubMed: Song 2021 - - Germline - - - - - Johan den Dunnen
+/+?, +/. 4 7 c.643C>T r.(?), r.535_652del p.(Arg215Ter), p.Asp179Valfs*23 ACMG pathogenic, pathogenic (recessive) g.46660525G>A g.46194853G>A in-frame skip exon 7 - POMGNT1_000139 1 Italian MDDGA3 (MEB) patient (com-het) and 1 MDDGA3 (MEB) patient (hom) with unknown ethnicity, 1 more item PubMed: Cavdarli 2023, PubMed: Saredi 2012 - rs386834034 Germline, SUMMARY record - - - - - Johan den Dunnen
+/., +?/. 3 7i c.652+1G>A r.spl, r.spl? p.? - likely pathogenic, likely pathogenic (recessive), pathogenic g.46660515C>T g.46194843C>T POMGNT1 mutation in intron 7 - POMGNT1_000044 probably c.652+1G>A (reference Godfrey et al.. 2007); heterozygous PubMed: Godfrey 2007, PubMed: Voglmeir 2011 - - Germline, SUMMARY record, Unknown -, ? - - - - Johan den Dunnen, Anne Polvi
-?/. 1 - c.653-9T>C r.(=) p.(=) - likely benign g.46660332A>G g.46194660A>G POMGNT1(NM_017739.3):c.653-9T>C - POMGNT1_000177 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 8 c.664G>A r.(?) p.(Gly222Arg) - VUS g.46660312C>T g.46194640C>T - - POMGNT1_000225 - PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
+/+?, +/., +?/. 6 8 c.667G>A r.(667g>a), r.(?) p.(Glu223Lys) - likely pathogenic, pathogenic, pathogenic (recessive) g.46660309C>T g.46194637C>T 667G>A;56G>A, 761G>A, POMGNT1 E223K - POMGNT1_000016 1 Japanese and 1 Belgian MDDGA3 (MEB) patient (both com-het), 1 more item PubMed: Song 2021, PubMed: Taniguchi 2003, PubMed: Manya 2003, PubMed: Voglmeir 2011 - - Germline, SUMMARY record, Unknown -, ? - - - - Johan den Dunnen
-/. 6 8 c.681A>G r.(?) p.(=), p.(?), p.(Lys227=) - benign g.46660295T>C g.46194623T>C POMGNT1(NM_001243766.2):c.681A>G (p.K227=), POMGNT1(NM_017739.3):c.681A>G (p.K227=) - POMGNT1_000045 other pathogenic change in several patients, VKGL data sharing initiative Nederland from website {DBsub-Emory}, PubMed: Godfrey 2007 - rs2292487 CLASSIFICATION record, Germline, Unknown - - - - - Johan den Dunnen, Madhuri Hegde, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen, VKGL-NL_AMC
+/., +?/. 2 8 c.703G>C r.(?) p.(Gly235Arg) - likely pathogenic (recessive), pathogenic g.46660273C>G g.46194601C>G - - POMGNT1_000154 - PubMed: Yis 2014 - - Germline, SUMMARY record - - - - - Johan den Dunnen, Anne Polvi
?/. 1 8 c.706G>A r.(?) p.(Asp236Asn) - VUS g.46660270C>T g.46194598C>T - - POMGNT1_000224 no second variant PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
?/. 1 9 c.758A>T r.(?) p.(Glu253Val) - VUS g.46660067T>A g.46194395T>A - - POMGNT1_000223 - PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
+?/. 1 - c.776_777del r.(?) p.(Thr259Argfs*14) - likely pathogenic g.46660051_46660052del - POMGNT1(NM_017739.4):c.776_777del (p.(Thr259Argfs*14)) - LURAP1_000039 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 2 9 c.788G>A r.(?) p.(Arg263His) - VUS g.46660037C>T g.46194365C>T - - POMGNT1_000222 no second variant PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
?/. 1 - c.790C>T r.(?) p.(Arg264Cys) - VUS g.46660035G>A g.46194363G>A - - POMGNT1_000248 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - rs369306680 Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
+/., +?/., -?/., ?/. 10 9 c.794G>A r.(?), r.794g>a p.(Arg265His), p.Arg265His - likely benign, likely pathogenic, likely pathogenic (recessive), pathogenic, VUS g.46660031C>T g.46194359C>T 935G>A, 935G>A (R265H), POMGNT1 R265H - POMGNT1_000019 conflicting interpretations of pathogenicity; 1 heterozygous, no homozygous; Clinindb (India), 4 more items PubMed: Bouchet 2007, PubMed: Narang 2020, Journal: Narang 2020, PubMed: Shenoy 2010, 3 more items - rs386834010 Germline, SUMMARY record, Unknown -, ?, yes 1/2795 individuals - - - Johan den Dunnen, Anne Polvi, Tom Winder, Mohammed Faruq
+/. 5 - c.794G>C r.(?) p.(Arg265Pro) - pathogenic, pathogenic (recessive) g.46660031C>G g.46194359C>G - - POMGNT1_000157 - PubMed: Fu 2017, PubMed: Song 2021 - - Germline - - - - - Johan den Dunnen, Xiaona Fu
+/. 1 - c.796C>T r.(?) p.(Arg266Trp) - pathogenic (recessive) g.46660029G>A g.46194357G>A - - POMGNT1_000267 - PubMed: Song 2021 - - Germline - - - - - Johan den Dunnen
?/. 1 9 c.797G>A r.(?) p.(Arg266Gln) - VUS g.46660028C>T g.46194356C>T - - POMGNT1_000221 no second variant PubMed: Nallamilli 2018 - - Germline - - - - - Madhuri Hegde
-/. 1 9 c.805_807del r.(?) p.(Cys269del) - benign g.46660019_46660021del g.46194347_46194349del - - POMGNT1_000047 - PubMed: Manya 2008 - - Germline - - - - - Johan den Dunnen
+/., +?/. 3 9 c.806G>A r.(?) p.(Cys269Tyr) - likely pathogenic, likely pathogenic (recessive), pathogenic g.46660019C>T g.46194347C>T 900G>A, POMGNT1 C269Y - POMGNT1_000048 protein concentrations of the expressed mutant rPOMGnT1 protein diminished; heterozygous PubMed: Taniguchi 2003, PubMed: Manya 2003, PubMed: Matsumoto 2005, PubMed: Voglmeir 2011 - - Germline, SUMMARY record, Unknown -, ? - - - - Johan den Dunnen, Anne Polvi
?/. 1 - c.811A>G r.(?) p.(Lys271Glu) - VUS g.46660014T>C - POMGNT1(NM_017739.4):c.811A>G (p.(Lys271Glu)) - LURAP1_000038 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.812A>G r.(?) p.(Lys271Arg) - VUS g.46660013T>C g.46194341T>C - - POMGNT1_000247 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - - Germline - 1/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
-?/. 1 - c.816T>C r.(?) p.(Val272=) - likely benign g.46660009A>G g.46194337A>G POMGNT1(NM_017739.3):c.816T>C (p.V272=) - LURAP1_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 4 9 c.839G>A r.(?) p.(Ser280Asn) - VUS g.46659986C>T g.46194314C>T POMGNT1(NM_001243766.1):c.839G>A (p.S280N) - POMGNT1_000220 no second variant, VKGL data sharing initiative Nederland PubMed: Nallamilli 2018 - - CLASSIFICATION record, Germline - - - - - Madhuri Hegde, VKGL-NL_Rotterdam
+/., ?/. 3 9 c.860T>G r.(?) p.(Ile287Ser) - pathogenic (recessive), VUS g.46659965A>C g.46194293A>C c.860T>G - POMGNT1_000250 2 more items PubMed: Panneman 2023, PubMed: Xu 2016, PubMed: Xu 2016 - - Germline, Unknown yes 1/309 unresolved RP cases - - - Johan den Dunnen, Daan Panneman
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