Unique variants in the HEXB gene

Information The variants shown are described using the NM_000521.3 transcript reference sequence.

65 entries on 1 page. Showing entries 1 - 65.
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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 _1_14_ c.-117_*113{0} r.0 p.0 - pathogenic (recessive) g.(?_73980969)_(74017113_?)del g.(?_74685144)_(74721288_?)del - - HEXB_000058 - PubMed: Granger 2022 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.-50449G>A r.(?) p.(=) - likely benign g.73930637G>A g.74634812G>A ENC1(NM_001256574.1):c.1674C>T (p.Y558=) - HEXB_000026 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.-49615G>A r.(?) p.(=) - likely benign g.73931471G>A g.74635646G>A ENC1(NM_001256574.1):c.840C>T (p.D280=) - HEXB_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.7C>T r.(?) p.(Leu3=) - likely benign g.73981092C>T - HEXB(NM_000521.3):c.7C>T (p.L3=) - HEXB_000049 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.39G>A r.(?) p.(Met13Ile) - likely benign g.73981124G>A g.74685299G>A HEXB(NM_000521.3):c.39G>A (p.M13I) - HEXB_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.52C>T r.(?) p.(=) - likely benign g.73981137C>T - HEXB(NM_000521.3):c.52C>T (p.L18=) - HEXB_000064 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/., +?/. 2 - c.170G>A r.(?) p.(Trp57Ter) - likely pathogenic (recessive), pathogenic g.73981255G>A g.74685430G>A - - HEXB_000019 ACMG PVS1, PM2, VKGL data sharing initiative Nederland PubMed: Charng 2016 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Nijmegen
-/. 2 - c.185= r.(=) p.(Ser62=) - benign g.73981270T>C g.74685445T>C HEXB(NM_000521.4):c.185T>C (p.L62S) - HEXB_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen, VKGL-NL_Nijmegen
-?/. 1 - c.189G>A r.(?) p.(Val63=) - likely benign g.73981274G>A g.74685449G>A HEXB(NM_000521.3):c.189G>A (p.V63=) - HEXB_000037 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 2 - c.214C>T r.(?) p.(Leu72Phe) - likely benign g.73981299C>T g.74685474C>T - - HEXB_000040 1 homozygous; Clinindb (India), 43 heterozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs147155126 Germline - 1/2795 individuals, 43/2795 individuals - - - Mohammed Faruq
-?/. 1 - c.258G>A r.(?) p.(Thr86=) - likely benign g.73981343G>A g.74685518G>A HEXB(NM_000521.4):c.258G>A (p.T86=) - HEXB_000028 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-/. 1 - c.276C>T r.(?) p.(Thr92=) - benign g.73981361C>T g.74685536C>T HEXB(NM_000521.3):c.276C>T (p.T92=) - HEXB_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.300-32C>T r.(=) p.(=) - benign g.73985121C>T - HEXB(NM_000521.3):c.300-32C>T - HEXB_000065 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-/. 2 - c.362A>G r.(?) p.(Lys121Arg) - benign g.73985215A>G - HEXB(NM_000521.3):c.362A>G (p.K121R) - HEXB_000056 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_Nijmegen
-?/., ?/. 2 - c.383T>G r.(?) p.(Leu128Arg) - likely benign, VUS g.73985236T>G g.74689411T>G HEXB(NM_000521.3):c.383T>G (p.L128R) - HEXB_000045 VKGL data sharing initiative Nederland PubMed: Fernandez-Marmiesse 2014 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Rotterdam
+/. 13 2i c.445+1G>A r.spl p.? - pathogenic g.73985299G>A g.74689474G>A - - HEXB_000011 combined HEX activity 0.0251, HEX activity 0.0118, HEX activity 0.0132, HEX activity 0.0158, 1 more item Oller Ramirez, SAG2017, Cordoba - - Germline - 118/132 case chromosomes, 9/200 controls - - - Johan den Dunnen
-?/. 1 - c.445+7A>G r.(=) p.(=) - likely benign g.73985305A>G g.74689480A>G HEXB(NM_000521.3):c.445+7A>G - HEXB_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.449C>A r.(?) p.(Thr150Asn) - likely benign g.73989467C>A - HEXB(NM_000521.3):c.449C>A (p.T150N) - HEXB_000057 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/. 1 - c.552T>G r.(?) p.(Tyr184Ter) - pathogenic g.73992558T>G - - - HEXB_000044 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 1 - c.558+1G>C r.spl? p.? - pathogenic g.73992565G>C g.74696740G>C - - HEXB_000021 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/., ?/. 24 5 c.619A>G r.(?) p.(Ile207Val) - benign, VUS g.73992881A>G g.74697056A>G HEXB 5:7402863 (hg18) het CM910221 23.1%, HEXB(NM_000521.4):c.619A>G (p.I207V) - HEXB_000001 non-causative, heterozygous, VKGL data sharing initiative Nederland PubMed: Bell 2011 - - CLASSIFICATION record, Germline ? - - - - Gerard C.P. Schaafsma, VKGL-NL_Groningen
?/. 1 - c.633A>T r.(?) p.(Arg211Ser) - VUS g.73992895A>T g.74697070A>T - - HEXB_000022 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 3 - c.715G>A r.(?) p.(Val239Ile) - VUS g.74001089G>A g.74705264G>A HEXB(NM_000521.3):c.715G>A (p.V239I, p.(Val239Ile)), HEXB(NM_001292004.2):c.40G>A (p.V14I) - HEXB_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen
+?/. 1 - c.771+5G>C r.spl? p.? - likely pathogenic g.74001150G>C g.74705325G>C - - HEXB_000041 1 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs727503959 Germline - 1/2792 individuals - - - Mohammed Faruq
+/. 1 7 c.782_785del r.(?) p.((Ser261Cysfs*12) - pathogenic g.74009341_74009344del g.74713516_74713519del 782_785delCTTT - HEXB_000012 not in 200 control individuals Oller Ramirez, SAG2017, Cordoba - - Germline - 1/132 case chromosomes - - - Johan den Dunnen
+/. 1 7 c.796T>G r.(?) p.(Tyr266Asp) - pathogenic g.74009355T>G g.74713530T>G - - HEXB_000013 combined HEX activity 0.0265 Oller Ramirez, SAG2017, Cordoba - - Germline - 1/132 case chromosomes - - - Johan den Dunnen
?/. 1 7 c.825del r.(?) p.(Ile275Metfs*32) - VUS g.74009384del g.74713559del - - HEXB_000002 - - - - Germline - - - - - Gerard C.P. Schaafsma
+/. 1 - c.826_829del r.(?) p.(Glu276MetfsTer30) - pathogenic g.74009385_74009388del - HEXB(NM_001292004.2):c.151_154delGAAT (p.E51Mfs*30) - HEXB_000038 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/. 1 - c.850C>T r.(?) p.(Arg284*) - pathogenic g.74009409C>T g.74713584C>T - - HEXB_000042 1 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs121907986 Germline - 1/2791 individuals - - - Mohammed Faruq
?/. 1 - c.851G>A r.(?) p.(Arg284Gln) - VUS g.74009410G>A g.74713585G>A - - HEXB_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 1 - c.902-2A>G r.spl? p.? - pathogenic g.74011333A>G g.74715508A>G - - HEXB_000024 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 3 8i c.1082+5G>A r.spl, r.spl? p.? ACMG pathogenic g.74011520G>A g.74715695G>A - - HEXB_000003 not in 200 control individuals, not in 200 control individuals; combined HEX activity 0.0251 Oller Ramirez, SAG2017, Cordoba, PubMed: Trujillano 2017 - - Germline - 2/132 case chromosomes - - - Johan den Dunnen, Daniel Trujillano
+/. 1 - c.1083-2del r.spl p.? - pathogenic g.74012410del g.74716585del 1083-2delA - HEXB_000048 - - - - Germline - - - - - Massoud Houshmand
+/., +?/. 2 9i c.1169+3_1169+10del r.spl, r.spl? p.? ACMG likely pathogenic, pathogenic g.74012501_74012508del g.74716676_74716683del HEXB, NM_000521.3, c.1169+3_1169+10del - HEXB_000004 homozygous PubMed: Alfares 2018, PubMed: Trujillano 2017 - - Germline, Unknown ? frequency in 1500 in-house samples: 0 - - - Daniel Trujillano
+/. 2 10i c.1242+1G>A r.spl p.? - pathogenic g.74014189G>A g.74718364G>A - - HEXB_000005 not in 200 control individuals Oller Ramirez, SAG2017, Cordoba - - Germline - 2/132 case chromosomes - - - Johan den Dunnen
+/., ?/. 3 - c.1250C>T r.(?) p.(Pro417Leu) - pathogenic, VUS g.74014629C>T g.74718804C>T HEXB(NM_001292004.1):c.575C>T (p.P192L), HEXB(NM_001292004.2):c.575C>T (p.P192L) - HEXB_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, VKGL-NL_VUmc
-/. 1 - c.1258A>G r.(?) p.(Ile420Val) - benign g.74014637A>G - HEXB(NM_000521.3):c.1258A>G (p.I420V) - HEXB_000053 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/. 1 10 c.1290dup r.(?) p.(Val404Cysfs*7) - pathogenic g.74014669dup g.74718844dup 1290dupT - HEXB_000006 - Oller Ramirez, SAG2017, Cordoba - - Germline - 1/132 case chromosomes - - - Johan den Dunnen
-?/., ?/. 2 - c.1338T>A r.(?) p.(Ser446=) - likely benign, VUS g.74014717T>A g.74718892T>A HEXB(NM_000521.4):c.1338T>A (p.(Ser446=)), HEXB(NM_001292004.1):c.663T>A (p.S221=) - HEXB_000043 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
-?/. 1 - c.1416C>T r.(?) p.(Gly472=) - likely benign g.74014795C>T g.74718970C>T HEXB(NM_001292004.1):c.741C>T (p.G247=) - HEXB_000031 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 12 c.1451G>A r.(?) p.(Gly484Glu) - pathogenic g.74016286G>A g.74720461G>A - - HEXB_000007 not in 200 control individuals Oller Ramirez, SAG2017, Cordoba - - Germline - 2/132 case chromosomes - - - Johan den Dunnen
+/. 6 12i c.1509-26G>A r.(=), r.1508_1509ins1509-24_1509-1, r.spl p.(=), p.?, p.Trp503_Pro504insGlyMetCysAspLeuAsnPheArg ACMG pathogenic, pathogenic (recessive) g.74016442G>A g.74720617G>A, NC_000005.10:g.74720617G>A HEXB(NM_000521.3):c.1509-26G>A, IVS12-26G>A - HEXB_000046 total enzyme deficiency, VKGL data sharing initiative Nederland PubMed: Dlott 1990, PubMed: Nakano 1989, PubMed: Dlott 1990 ClinVar-527971 rs201580118 CLASSIFICATION record, Germline yes - - - - Johan den Dunnen, VKGL-NL_Utrecht, VKGL-NL_Nijmegen, Marketa Wayhelova
+/. 1 - c.1513C>T r.(?) p.(Arg505Trp) - pathogenic (recessive) g.74016472C>T g.74720647C>T - - HEXB_000059 - PubMed: Granger 2022 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.1515G>A r.(?) p.(Arg505=) - likely benign g.74016474G>A g.74720649G>A HEXB(NM_001292004.2):c.840G>A (p.R280=) - HEXB_000032 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
?/. 1 - c.1525G>A r.(?) p.(Val509Ile) - VUS g.74016484G>A - HEXB(NM_001292004.1):c.850G>A (p.V284I) - HEXB_000050 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.1538T>C r.(?) p.(Leu513Pro) - VUS g.74016497T>C g.74720672T>C - - HEXB_000025 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 1 - c.1550_1553dup r.(?) p.(Asp518GlufsTer8) - pathogenic g.74016509_74016512dup - HEXB(NM_001292004.2):c.875_878dupAAGA (p.D293Efs*8) - HEXB_000039 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/. 1 13 c.1578T>G r.(?) p.(Tyr526*) ACMG pathogenic g.74016537T>G g.74720712T>G - - HEXB_000066 - - ClinVar-1071217 rs1460801055 Germline yes - - - - Marketa Wayhelova
+/. 1 13 c.1597C>T r.(?) p.(Arg533Cys) - pathogenic g.74016556C>T g.74720731C>T - - HEXB_000008 not in 200 control individuals Oller Ramirez, SAG2017, Cordoba - - Germline - 1/132 case chromosomes - - - Johan den Dunnen
+/. 1 13 c.1601G>A r.(?) p.Cys534Tyr) - pathogenic g.74016560G>A g.74720735G>A - - HEXB_000009 not in 200 control individuals Oller Ramirez, SAG2017, Cordoba - - Germline - 1/132 case chromosomes - - - Johan den Dunnen
+/. 1 13i_14 c.1614-16_1615dup r.[1614-16_1615dup,=] p.[Glu538_Arg539insLeuHisValIleTyrArg,=] - pathogenic (recessive) g.74016927_74016944dup g.74721102_74721119dup - - HEXB_000067 - PubMed: Dlott 1990 - - Germline - - - - - Johan den Dunnen
+/. 1 14 c.1615C>T r.(?) p.(Arg539Cys) - pathogenic g.74016944C>T g.74721119C>T - - HEXB_000010 combined HEX activity 0.0265 Oller Ramirez, SAG2017, Cordoba - - Germline - 1/132 case chromosomes - - - Johan den Dunnen
-?/., ?/. 2 - c.1625C>A r.(?) p.(Ala542Asp) - likely benign, VUS g.74016954C>A - HEXB(NM_000521.4):c.1625C>A (p.(Ala542Asp)), HEXB(NM_001292004.1):c.950C>A (p.A317D) - HEXB_000051 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
-?/. 2 - c.1627G>A r.(?) p.(Ala543Thr) - likely benign g.74016956G>A g.74721131G>A HEXB(NM_001292004.2):c.952G>A (p.A318T) - HEXB_000033 17 heterozygous, no homozygous; Clinindb (India), VKGL data sharing initiative Nederland PubMed: Narang 2020, Journal: Narang 2020 - rs121907984 CLASSIFICATION record, Germline - 17/2795 individuals - - - VKGL-NL_Groningen, Mohammed Faruq
-?/. 1 - c.1670A>G r.(?) p.(*557=) - likely benign g.74016999A>G - HEXB(NM_001292004.1):c.995A>G (p.*332=) - HEXB_000052 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.*82_*83del r.(=) p.(=) - benign g.74017082_74017083del - GFM2(NM_032380.5):c.*398_*399delCA, HEXB(NM_000521.3):c.*82_*83delGT - HEXB_000063 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. 1 - c.*496C>T r.(=) p.(=) - VUS g.74017496C>T - GFM2(NM_032380.5):c.2324G>A (p.(Arg775Lys)) - HEXB_000068 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/., ?/. 2 - c.*533T>C r.(=) p.(=) - likely benign, VUS g.74017533T>C - GFM2(NM_001281302.1):c.2383A>G (p.M795V), GFM2(NM_032380.5):c.2287A>G (p.M763V) - HEXB_000054 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen
?/. 1 - c.*1233dup r.(?) p.(=) - VUS g.74018233dup - GFM2(NM_032380.5):c.2182dupA (p.I728Nfs*66) - HEXB_000047 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+?/. 1 - c.*1610G>A r.(=) p.(=) - likely pathogenic g.74018610G>A - GFM2(NM_001281302.2):c.2125-224C>T - HEXB_000060 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
?/. 1 - c.*4500T>C r.(=) p.(=) - VUS g.74021500T>C - GFM2(NM_032380.5):c.1993A>G (p.M665V) - HEXB_000061 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
?/. 1 - c.*18829C>T r.(=) p.(=) - VUS g.74035829C>T - GFM2(NM_001281302.1):c.1160G>A (p.R387H) - HEXB_000055 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.*26583C>T r.(=) p.(=) - VUS g.74043583C>T - GFM2(NM_032380.5):c.542G>A (p.R181K) - HEXB_000062 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. 1 - c.*29495A>G r.(=) p.(=) - likely benign g.74046495A>G g.74750670A>G GFM2(NM_001281302.1):c.527-3T>C - HEXB_000035 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 - c.*30274G>A r.(=) p.(=) - pathogenic g.74047274G>A g.74751449G>A GFM2(NM_001281302.2):c.445C>T (p.R149*) - HEXB_000036 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
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