All variants in the CHKB gene

This database is one of the gene variant databases from the Leiden Muscular Dystrophy pages
Information The variants shown are described using the NM_005198.4 transcript reference sequence.

85 entries on 1 page. Showing entries 1 - 85.
Legend   How to query  

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.-645576_*3834666del r.0? p.0? - pathogenic g.47182944_51666786del - - - ALG12_000022 mosaicism, hemizygous in 0.56 cells PubMed: DDDS 2015, Journal: DDDS 2015 - - Somatic - - - - - Johan den Dunnen
?/. - c.-74_-55del r.(=) p.(=) - VUS g.51021271_51021290del g.50582842_50582861del - - CHKB_000012 - - - rs144647670 Germline - - - - - Andreas Laner
-?/. - c.38C>T r.(?) p.(Ala13Val) - likely benign g.51021173G>A - CHKB(NM_005198.4):c.38C>T (p.A13V) - CHKB_000052 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.64G>A r.(?) p.(Gly22Ser) - likely benign g.51021147C>T g.50582718C>T - - CHKB_000042 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 1 c.116C>A r.(?) p.(Ser39*) - pathogenic g.51021095G>T g.50582666G>T - - CHKB_000002 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0002 - - Germline - - - - - Johan den Dunnen
+/. 1 c.116C>A r.(?) p.(Ser39*) - pathogenic g.51021095G>T g.50582666G>T - - CHKB_000002 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0002 - - Germline - - - - - Johan den Dunnen
-?/. - c.149A>G r.(?) p.(Tyr50Cys) - likely benign g.51021062T>C - CHKB(NM_005198.4):c.149A>G (p.(Tyr50Cys)) - CHKB_000053 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. - c.149A>G r.(?) p.(Tyr50Cys) - VUS g.51021062T>C - - - CHKB_000053 - PubMed: Chen 2022 - - Germline/De novo (untested) - - - - - Johan den Dunnen
-?/. - c.214T>C r.(?) p.(Tyr72His) - likely benign g.51020997A>G g.50582568A>G - - CHKB_000041 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. - c.248dup r.(?) p.(Arg84ProfsTer126) - pathogenic g.51020764dup - - - CHKB_000054 - - - - Unknown - - - - - MobiDetails
-?/. - c.249C>T r.(?) p.(Phe83=) - likely benign g.51020762G>A g.50582333G>A CHKB(NM_005198.4):c.249C>T (p.F83=) - CHKB_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.249C>T r.(?) p.(Phe83=) - likely benign g.51020762G>A - CHKB(NM_005198.4):c.249C>T (p.F83=) - CHKB_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. - c.263C>T r.(?) p.(Pro88Leu) - VUS g.51020748G>A - CHKB(NM_005198.4):c.263C>T (p.P88L) - CHKB_000046 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.333+6G>A r.(=) p.(=) - likely benign g.51020672C>T g.50582243C>T CHKB(NM_005198.4):c.333+6G>A - CHKB_000045 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. - c.333+10G>T r.(=) p.(=) - benign g.51020668C>A g.50582239C>A - - CHKB_000031 dbSNP: frequency 40-60% - - rs86337 Germline - frequency 40-60% - - - Andreas Laner
-/. - c.333+10G>T r.(=) p.(=) - benign g.51020668C>A g.50582239C>A CHKB(NM_005198.5):c.333+10G>T, CHKB-CPT1B(NR_027928.2):n.551+10G>T - CHKB_000031 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. - c.382G>T r.(?) p.(Glu128*) ACMG pathogenic (recessive) g.51020243C>A g.50581814C>A - - CHKB_000057 ACMG PVS1, PS4, PM2, PM3, PP1, PP3 PubMed: Ghasemi 2024 SCV000923677 - Germline - - - - - Johan den Dunnen
+/. - c.419del r.(?) p.(Pro140Glnfs*14) - pathogenic g.51020208del - CHKB(NM_005198.4):c.419delC (p.P140Qfs*14) - CHKB_000048 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. - c.419del r.(?) p.(Pro140Glnfs*14) - pathogenic g.51020208del - CHKB(NM_005198.4):c.419delC (p.P140Qfs*14) - CHKB_000048 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 3_9i c.(422_(1031+1_1032-1)del) r.del p.fs* - pathogenic g.((51017937_51018155)_51020203)del - - - CHKB_000000 deletion from exon 3 to intron 9 PubMed: Sher 2006 - - animal model - - - - - Johan den Dunnen
?/. - c.439T>G r.(?) p.(Tyr147Asp) - VUS g.51020186A>C g.50581757A>C CHKB(NM_005198.5):c.439T>G (p.Y147D) - CHKB_000036 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. - c.457T>C r.(?) p.(Leu153=) - likely benign g.51019973A>G g.50581544A>G CHKB(NM_005198.4):c.457T>C (p.L153=) - CHKB_000040 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/. 3 c.458dup r.(?) p.(Leu153Phefs*57) - pathogenic g.51019973dup g.50581544dup - - CHKB_000003 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0003 - - Germline - - - - - Johan den Dunnen
+/. 3 c.458dup r.(?) p.(Leu153Phefs*57) - pathogenic g.51019973dup g.50581544dup - - CHKB_000003 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0003 - - Germline - - - - - Johan den Dunnen
+?/. - c.475C>T r.(?) p.(Arg159Ter) - likely pathogenic g.51019955G>A - CHKB(NM_005198.5):c.475C>T (p.R159*) - CHKB_000035 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. - c.501T>G r.(?) p.(Ile167Met) - likely benign g.51019929A>C g.50581500A>C CHKB(NM_005198.5):c.501T>G (p.I167M) - CHKB_000020 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. 4 c.517_529del r.(?) p.(Gln173TrpfsTer20) - pathogenic g.51019901_51019913del g.50581472_50581484del c.517_529delCAATTTCATGGCA - CHKB_000049 Novel variant (2021) PubMed: Karthikeyan 2024 - - Germline/De novo (untested) - - - - - Lakshmi Bremadesam
+/. 4 c.554_562del r.(?) p.(Pro185_Trp187del) - pathogenic g.51019870_51019878del g.50581441_50581449del - - CHKB_000008 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
+/. 4 c.554_562del r.(?) p.(Pro185_Trp187del) - pathogenic g.51019870_51019878del g.50581441_50581449del - - CHKB_000008 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
+/. 4 c.554_562del r.(?) p.(Pro185_Trp187del) - NA g.51019870_51019878del g.50581441_50581449del - - CHKB_000008 cloned, purified enzyme activity <30% PubMed: Mitsuhashi 2011 - - In vitro (cloned) - - - - - Johan den Dunnen
+/. - c.598del r.(?) p.(Gln200ArgfsTer11) - pathogenic (recessive) g.51019074del g.50580645del - - CHKB_000019 - PubMed: Westra 2019 - - Germline - - - - - Johan den Dunnen
+/. 5 c.611dup r.(?) p.(Thr205Asnfs*5) - pathogenic g.51019064dup g.50580635dup - - CHKB_000004 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
+/. 5 c.611dup r.(?) p.(Thr205Asnfs*5) - pathogenic g.51019064dup g.50580635dup - - CHKB_000004 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
-?/. - c.670A>C r.(?) p.(Asn224His) - likely benign g.51019001T>G g.50580572T>G CHKB(NM_005198.4):c.670A>C (p.(Asn224His), p.N224H) - CHKB_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. - c.670A>C r.(?) p.(Asn224His) - likely benign g.51019001T>G - CHKB(NM_005198.4):c.670A>C (p.(Asn224His), p.N224H) - CHKB_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 5i c.677+1G>A r.spl? p.? - pathogenic g.51018993C>T g.50580564C>T - - CHKB_000009 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0005 - - Germline - - - - - Johan den Dunnen
+/. 5i c.677+1G>A r.spl? p.? - pathogenic g.51018993C>T g.50580564C>T - - CHKB_000009 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0005 - - Germline - - - - - Johan den Dunnen
+/. 5i c.677+1G>A r.spl? p.? - pathogenic g.51018993C>T g.50580564C>T - - CHKB_000009 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0005 - - Germline - - - - - Johan den Dunnen
+/. 5i c.677+1G>A r.spl? p.? - pathogenic g.51018993C>T g.50580564C>T - - CHKB_000009 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0005 - - Germline - - - - - Johan den Dunnen
+/. 5i c.677+1G>A r.spl? p.? - pathogenic g.51018993C>T g.50580564C>T - - CHKB_000009 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0005 - - Germline - - - - - Johan den Dunnen
+/. 5i c.677+1G>A r.spl? p.? - pathogenic g.51018993C>T g.50580564C>T - - CHKB_000009 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0005 - - Germline - - - - - Johan den Dunnen
-/. - c.678-66G>A r.(=) p.(=) - benign g.51018911C>T g.50580482C>T - - CHKB_000030 - - - rs2269382 Germline - - - - - Andreas Laner
+?/. - c.682_683dup r.(?) p.(Leu228Phefs*3) - likely pathogenic (recessive) g.51018840_51018841dup g.50580411_50580412dup 682_683insTT - CHKB_000043 no variant 2nd allele PubMed: Park 2017 - - Germline - 1/209 cases - - - Johan den Dunnen
?/. - c.722A>G r.(?) p.(Asn241Ser) - VUS g.51018801T>C - - - CHKB_000055 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. - c.788_790del r.(?) p.(Val263del) - VUS g.51018650_51018652del - CHKB(NM_005198.5):c.788_790delTGG (p.V263del) - CHKB_000047 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. 7 c.810T>A r.(?) p.(Tyr270*) - pathogenic g.51018627A>T g.50580198A>T - - CHKB_000001 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0001 - - Germline - - - - - Johan den Dunnen
+/. 7 c.810T>A r.(?) p.(Tyr270*) - pathogenic g.51018627A>T g.50580198A>T - - CHKB_000001 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0001 - - Germline - - - - - Johan den Dunnen
+/. 7 c.810T>A r.(?) p.(Tyr270*) - pathogenic g.51018627A>T g.50580198A>T - - CHKB_000001 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0001 - - Germline - - - - - Johan den Dunnen
+/. 7 c.810T>A r.(?) p.(Tyr270*) - pathogenic g.51018627A>T g.50580198A>T - - CHKB_000001 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0001 - - Germline - - - - - Johan den Dunnen
+/. - c.817del r.(?) p.(Arg273GlyfsTer68) - pathogenic g.51018620del g.50580191del - - CHKB_000033 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. - c.818+40T>C r.(=) p.(=) - benign g.51018579A>G g.50580150A>G - - CHKB_000029 dbSNP: frequency up to 50% - - rs140514 Germline - frequency up to 50% - - - Andreas Laner
+/. 8 c.847G>A r.(?) p.(Glu283Lys) - pathogenic g.51018483C>T g.50580054C>T - - CHKB_000006 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
+/. 8 c.847G>A r.(?) p.(Glu283Lys) - pathogenic g.51018483C>T g.50580054C>T - - CHKB_000006 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
+/. 8 c.847G>A r.(?) p.(Glu283Lys) - NA g.51018483C>T g.50580054C>T - - CHKB_000006 cloned, purified enzyme activity <30% PubMed: Mitsuhashi 2011 - - In vitro (cloned) - - - - - Johan den Dunnen
+/. 8 c.852_859del r.(?) p.(Trp284*) - pathogenic g.51018473_51018480del g.50580044_50580051del - - CHKB_000011 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
+/. 8 c.852_859del r.(?) p.(Trp284*) - pathogenic g.51018473_51018480del g.50580044_50580051del - - CHKB_000011 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
-?/. - c.902C>T r.(?) p.(Thr301Ile) - likely benign g.51018428G>A g.50579999G>A CHKB(NM_005198.5):c.902C>T (p.T301I) - CHKB_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. 8 c.922C>T r.(?) p.(Gln308*) - pathogenic g.51018408G>A g.50579979G>A - - CHKB_000005 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0004 - - Germline - - - - - Johan den Dunnen
+/. 8 c.922C>T r.(?) p.(Gln308*) - pathogenic g.51018408G>A g.50579979G>A - - CHKB_000005 not in 210 control chromosomes PubMed: Mitsuhashi 2011, OMIM:var0004 - - Germline - - - - - Johan den Dunnen
-?/. - c.980C>T r.(?) p.(Ser327Phe) - likely benign g.51018207G>A g.50579778G>A - - CHKB_000039 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. - c.983A>G r.(?) p.(Gln328Arg) - likely benign g.51018204T>C g.50579775T>C CHKB(NM_005198.4):c.983A>G (p.Q328R) - CHKB_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. - c.983A>G r.(?) p.(Gln328Arg) - likely benign g.51018204T>C - CHKB(NM_005198.4):c.983A>G (p.Q328R) - CHKB_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.1008A>T r.(?) p.(Glu336Asp) - likely benign g.51018179T>A g.50579750T>A CHKB(NM_005198.4):c.1008A>T (p.E336D) - CHKB_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+?/. - c.1031G>A r.(?) p.(Arg344Gln) - likely pathogenic g.51018156C>T - CHKB(NM_005198.5):c.1031G>A (p.R344Q) - CHKB_000051 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/. 9i c.1031+1G>A r.[819_1031del, 928_1031del, 678_1031del, 968_1031del] p.[Gly274_Arg344del, Leu310Valfs*84, Lys227_Arg344del, Glu324Metfs*22] - pathogenic g.51018155C>T g.50579726C>T - - CHKB_000010 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
+/. 9i c.1031+1G>A r.[819_1031del, 928_1031del, 678_1031del, 968_1031del] p.[Gly274_Arg344del, Leu310Valfs*84, Lys227_Arg344del, Glu324Metfs*22] - pathogenic g.51018155C>T g.50579726C>T - - CHKB_000010 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
+/. 9i c.1031+1G>A r.spl? p.? - pathogenic g.51018155C>T g.50579726C>T - - CHKB_000010 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
+/. 9i c.1031+1G>A r.spl? p.? - pathogenic g.51018155C>T g.50579726C>T - - CHKB_000010 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
?/. - c.1031+3G>C r.spl? p.? - VUS g.51018153C>G g.50579724C>G CHKB(NM_005198.4):c.1031+3G>C (p.?) - CHKB_000032 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.1032-72A>G r.(=) p.(=) - VUS g.51018008T>C g.50579579T>C - - CHKB_000028 - - - - Germline - - - - - Andreas Laner
?/. - c.1032-71C>T r.(=) p.(=) - VUS g.51018007G>A g.50579578G>A - - CHKB_000027 - - - - Germline - - - - - Andreas Laner
-/. - c.1113+61T>C r.(=) p.(=) - benign g.51017794A>G g.50579365A>G - - CHKB_000026 - - - rs762676 Germline - - - - - Andreas Laner
-/. - c.1114-29C>T r.(=) p.(=) - benign g.51017713G>A g.50579284G>A - - CHKB_000025 - - - rs762677 Germline - - - - - Andreas Laner
+?/. - c.1114-11_1133del r.spl? p.? - likely pathogenic g.51017669_51017699del - CHKB(NM_005198.5):c.1114-11_1133delTCCTTCCCTAGGACTATGCCCAGTCTCGGTT - CHKB_000050 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/. 11 c.1130G>T r.(?) p.(Arg377Leu) - pathogenic g.51017668C>A g.50579239C>A - - CHKB_000007 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
+/. 11 c.1130G>T r.(?) p.(Arg377Leu) - pathogenic g.51017668C>A g.50579239C>A - - CHKB_000007 not in 210 control chromosomes PubMed: Mitsuhashi 2011 - - Germline - - - - - Johan den Dunnen
+/. 11 c.1130G>T r.(?) p.(Arg377Leu) - NA g.51017668C>A g.50579239C>A - - CHKB_000007 cloned, purified enzyme activity <30% PubMed: Mitsuhashi 2011 - - In vitro (cloned) - - - - - Johan den Dunnen
?/. - c.*96G>C r.(=) p.(=) - VUS g.51017514C>G g.50579085C>G - - CHKB_000021 - - - - Germline - - - - - Yu Sun
-/. - c.*134T>C r.(=) p.(=) - benign g.51017476A>G g.50579047A>G - - CHKB_000024 - - - rs1056964 Germline - - - - - Andreas Laner
-/. - c.*216C>G r.(=) p.(=) - benign g.51017394G>C g.50578965G>C - - CHKB_000023 - - - rs3180872 Germline - - - - - Andreas Laner
-/. - c.*257A>G r.(=) p.(=) - benign g.51017353T>C g.50578924T>C - - CHKB_000022 - - - rs5770917 Germline - - - - - Andreas Laner
-/. - c.*1250C>T r.(=) p.(=) - benign g.51016360G>A g.50577931G>A - - CHKB_000044 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. - c.*2129C>T r.(=) p.(=) - benign g.51015481G>A g.50577052G>A - - CHKB_000038 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. - c.*4756C>T r.(=) p.(=) - benign g.51012854G>A g.50574425G>A CPT1B(NM_152245.3):c.886-6C>T - CHKB_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-/. - c.*4756C>T r.(=) p.(=) - benign g.51012854G>A g.50574425G>A CPT1B(NM_152245.3):c.886-6C>T - CHKB_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
Legend   How to query  


Screenscraping/webscraping (downloading large amounts of data using scripts) is strictly prohibited.
Use our APIs to retrieve data.