All variants in the BCKDHB gene

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

96 entries on 1 page. Showing entries 1 - 96.
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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.*109G>A r.(?) p.(=) - benign g.81055792G>A g.80346075G>A - - BCKDHB_000064 - Journal: Jaradat 2016 - rs15062 Germline - - - - - Johan den Dunnen
-/. - c.*120C>T r.(?) p.(=) - benign g.81055803C>T g.80346086C>T - - BCKDHB_000065 - Journal: Jaradat 2016 - rs10864 Germline - - - - - Johan den Dunnen
-?/. - c.10G>A r.(?) p.(Val4Ile) - likely benign g.80816420G>A - BCKDHB(NM_183050.4):c.10G>A (p.V4I) - BCKDHB_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.23C>T r.(?) p.(Ala8Val) - VUS g.80816433C>T g.80106716C>T - - BCKDHB_000021 - PubMed: Taylor 2014 - - Germline - - - - - Johan den Dunnen
-?/. - c.51A>G r.(?) p.(Ala17=) - likely benign g.80816461A>G g.80106744A>G BCKDHB(NM_183050.4):c.51A>G (p.A17=) - BCKDHB_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. - c.51A>G r.(?) p.(Ala17=) - benign g.80816461A>G g.80106744A>G BCKDHB(NM_183050.4):c.51A>G (p.A17=) - BCKDHB_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. - c.79C>T r.(?) p.(Pro27Ser) - VUS g.80816489C>T g.80106772C>T BCKDHB(NM_000056.3):c.79C>T (p.(Pro27Ser)) - BCKDHB_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 c.152del r.(?) p.(Val51Glyfs*21) ACMG pathogenic (recessive) g.80816562del g.80106845del - - BCKDHB_000033 Bashyam 2012:22593002, Rodríguez-Pombo 2006:16786533 - ClinVar-527129 rs867612284 Germline yes - - - - Miriam Erandi Reyna-Fabián
+/. 1 c.163_166dup r.(?) p.(Phe56TyrfsTer29) - pathogenic (recessive) g.80816573_80816576dup g.80106856_80106859dup 163_166dupACTT - BCKDHB_000040 no enzyme activity PubMed: Henneke 2003, Journal: Henneke 2003 - - Germline - - - - - Johan den Dunnen
?/. - c.172C>T r.(?) p.(Pro58Ser) - VUS g.80816582C>T - BCKDHB(NM_183050.4):c.172C>T (p.P58S) - BCKDHB_000022 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. - c.(?_197-51)_(742+51_?)dup r.? p.? ACMG VUS g.(?_80837213)_(80881158_?)dup g.(?_80127496)_(80171441_?)dup 197-51_742+51dup - BCKDHB_000028 - - - - Germline ? - - - - Zeinab Sayed Abdelkhalek
-/. - c.197-25A>G r.(?) p.(=) - benign g.80837239A>G g.80127522A>G - - BCKDHB_000041 - Journal: Jaradat 2016 - rs9448893 Germline - - - - - Johan den Dunnen
-?/. - c.197-11del r.(=) p.(=) - likely benign g.80837253del g.80127536del BCKDHB(NM_183050.4):c.197-11delG - BCKDHB_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. - c.197-11G>T r.(=) p.(=) - benign g.80837253G>T g.80127536G>T BCKDHB(NM_000056.4):c.197-11G>T, BCKDHB(NM_183050.4):c.197-11G>T - BCKDHB_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. - c.197-11G>T r.(=) p.(=) - benign g.80837253G>T g.80127536G>T BCKDHB(NM_000056.4):c.197-11G>T, BCKDHB(NM_183050.4):c.197-11G>T - BCKDHB_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-/. - c.197-11G>T r.(=) p.(=) - benign g.80837253G>T g.80127536G>T BCKDHB(NM_000056.4):c.197-11G>T, BCKDHB(NM_183050.4):c.197-11G>T - BCKDHB_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. - c.197-11G>T r.(?) p.(=) - benign g.80837253G>T g.80127536G>T - - BCKDHB_000004 - Journal: Jaradat 2016 - rs9448894 Germline - - - - - Johan den Dunnen
-?/. - c.197-10A>T r.(=) p.(=) - likely benign g.80837254A>T g.80127537A>T BCKDHB(NM_183050.4):c.197-10A>T - BCKDHB_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. - c.197-2A>G r.spl p.? - pathogenic g.80837262A>G g.80127545A>G NM_183050.4:c.197-2A>G - BCKDHB_000026 - PubMed: Chuan 2022 - - Germline - - - - - Johan den Dunnen
-/. 2 c.207G>A r.(?) p.(Gln69=) - benign g.80837274G>A g.80127557G>A - - BCKDHB_000039 - Journal: Jaradat 2016 - - Germline - - - - - Johan den Dunnen
+/. - c.221del r.(?) p.(Phe74Serfs*4) ACMG pathogenic g.80837288del g.80127571del 218delT - BCKDHB_000027 ACMG PVS1,PM2,PM3,PM4 PubMed: Chuan 2022 - - Germline - - - - - Johan den Dunnen
?/. - c.238G>A r.(?) p.(Ala80Thr) - VUS g.80837305G>A - BCKDHB(NM_183050.4):c.238G>A (p.(Ala80Thr)) - BCKDHB_000029 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. 2i c.274+46G>A r.(?) p.(=) - benign g.80837387G>A g.80127670G>A - - BCKDHB_000037 - Journal: Jaradat 2016 - - Germline - - - - - Johan den Dunnen
-/. 2i c.274+54G>A r.(?) p.(=) - benign g.80837395G>A g.80127678G>A - - BCKDHB_000038 - Journal: Jaradat 2016 - - Germline - - - - - Johan den Dunnen
+?/. - c.281_291del r.(?) p.(Phe94CysfsTer8) - likely pathogenic g.80838884_80838894del - BCKDHB(NM_183050.4):c.281_291delTTGGTGAAGAT (p.F94Cfs*8) - BCKDHB_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/. 3 c.288del r.(?) p.(Asp97MetfsTer133) - pathogenic (recessive) g.80838891del g.80129174del 287_287delA - BCKDHB_000042 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - - Germline - 4/66 alleles MSUD - - - Johan den Dunnen
+/. 3 c.288del r.(?) p.(Asp97MetfsTer133) - pathogenic (recessive) g.80838891del g.80129174del 287_287delA - BCKDHB_000042 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - - Germline - 4/66 alleles MSUD - - - Johan den Dunnen
+?/. - c.302G>A r.(?) p.(Gly101Asp) - likely pathogenic g.80838905G>A g.80129188G>A - - BCKDHB_000017 2 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs398124571 Germline - 2/2795 individuals - - - Mohammed Faruq
+/. 3 c.331C>T r.(?) p.(Arg111Ter) - pathogenic (recessive) g.80838934C>T g.80129217C>T - - BCKDHB_000043 - PubMed: Flaschker 2007, Journal: Flaschker 2007 - - Germline - - - - - Johan den Dunnen
?/. - c.332G>A r.(?) p.(Arg111Gln) - VUS g.80838935G>A g.80129218G>A BCKDHB(NM_183050.4):c.332G>A (p.R111Q) - BCKDHB_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 4 c.368C>T r.(?) p.(Pro123Leu) - pathogenic (recessive) g.80877419C>T g.80167702C>T - - BCKDHB_000044 - PubMed: Flaschker 2007, Journal: Flaschker 2007 - - Germline - - - - - Johan den Dunnen
+/. 4 c.389T>G r.(?) p.(Val130Gly) - pathogenic (recessive) g.80877440T>G g.80167723T>G - - BCKDHB_000045 - PubMed: Flaschker 2007, Journal: Flaschker 2007 - - Germline - - - - - Johan den Dunnen
+?/. - c.397G>A r.(?) p.(Gly133Arg) - likely pathogenic g.80877448G>A - - - BCKDHB_000025 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 4 c.403G>A r.(?) p.(Gly135Arg) - pathogenic (recessive) g.80877454G>A g.80167737G>A - - BCKDHB_000046 enzyme activity <0.05 PubMed: Henneke 2003, Journal: Henneke 2003 - - Germline - - - - - Johan den Dunnen
+/. 4 c.410C>T r.(?) p.(Ala137Val) - pathogenic (recessive) g.80877461C>T g.80167744C>T - - BCKDHB_000047 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - rs776631396 Germline - 2/66 alleles MSUD - - - Johan den Dunnen
+/. 4 c.475C>A r.(?) p.(Gln159Lys) - pathogenic (recessive) g.80877526C>A g.80167809C>A - - BCKDHB_000048 no enzyme activity PubMed: Henneke 2003, Journal: Henneke 2003 - - Germline - - - - - Johan den Dunnen
+/. 5 c.502C>T r.(?) p.(Arg168Cys) - pathogenic (recessive) g.80878616C>T g.80168899C>T - - BCKDHB_000049 - PubMed: Flaschker 2007, Journal: Flaschker 2007 - - Germline - - - - - Johan den Dunnen
+/. 5 c.564T>A r.(?) p.(Cys188*) ACMG pathogenic (recessive) g.80878678T>A g.80168961T>A - - BCKDHB_000032 - - ClinVar-580585 rs774306610 Germline yes - - - - Miriam Erandi Reyna-Fabián
+/. 5 c.564T>A r.(?) p.(Cys188*) ACMG pathogenic (recessive) g.80878678T>A g.80168961T>A - - BCKDHB_000032 Bashyam 2012:22593002, Gorzelany 2009:22593002, Rodríguez-Pombo 2006:16786533 - ClinVar-580585 rs774306610 Germline yes - - - - Miriam Erandi Reyna-Fabián
+/. 5 c.595_596del r.(?) p.(Pro200Ter) - pathogenic (recessive) g.80878709_80878710del g.80168992_80168993del 595_596delAG - BCKDHB_000009 enzyme activity <0.05 PubMed: Henneke 2003, Journal: Henneke 2003 - - Germline - - - - - Johan den Dunnen
+/. 5 c.595_596del r.(?) p.(Pro200Ter) - pathogenic (recessive) g.80878709_80878710del g.80168992_80168993del 595_596delAG - BCKDHB_000009 - PubMed: Henneke 2003, Journal: Henneke 2003, PubMed: Flaschker 2007 - - Germline - - - - - Johan den Dunnen
+/. 5 c.598C>G r.(?) p.(Pro200Ala) - pathogenic (recessive) g.80878712C>G g.80168995C>G - - BCKDHB_000050 - PubMed: Flaschker 2007, Journal: Flaschker 2007 - - Germline - - - - - Johan den Dunnen
?/. - c.633G>C r.(?) p.(Lys211Asn) - VUS g.80878747G>C g.80169030G>C BCKDHB(NM_183050.4):c.633G>C (p.K211N) - BCKDHB_000020 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 6 c.665A>G r.(?) p.(Lys222Arg) - pathogenic (recessive) g.80881030A>G g.80171313A>G - - BCKDHB_000051 - PubMed: Henneke 2003, Journal: Henneke 2003 - - Germline - - - - - Johan den Dunnen
+?/. 6 c.667G>C r.(?) p.(Gly223Arg) ACMG likely pathogenic (recessive) g.80881032G>C g.80171315G>C - - BCKDHB_000034 Variant classified as per ACMG guidelines (Richards et al 2015) and to the recently developed ACMG scoring system (Tavtigian et al 2020). - - rs1248040495 Germline yes - - - - Miriam Erandi Reyna-Fabián
+/. - c.716A>G r.(?) p.(Glu239Gly) - pathogenic (recessive) g.80881081A>G g.80171364A>G - - BCKDHB_000052 - PubMed: Jaafar 2013, Journal: Jaafar 2013 - - Germline - - - - - Johan den Dunnen
+/. - c.716A>G r.(?) p.(Glu239Gly) - pathogenic (recessive) g.80881081A>G g.80171364A>G - - BCKDHB_000052 - PubMed: Jaafar 2013, Journal: Jaafar 2013 - - Germline - - - - - Johan den Dunnen
+/. 6 c.721A>T r.(?) p.(Lys241Ter) - pathogenic (recessive) g.80881086A>T g.80171369A>T - - BCKDHB_000053 enzyme activity <0.05 PubMed: Henneke 2003, Journal: Henneke 2003 - - Germline - - - - - Johan den Dunnen
+/. 6 c.721A>T r.(?) p.(Lys241Ter) - pathogenic (recessive) g.80881086A>T g.80171369A>T - - BCKDHB_000053 enzyme activity 0.05 PubMed: Henneke 2003, Journal: Henneke 2003 - - Germline - - - - - Johan den Dunnen
+/. - c.722_731dup r.(?) p.(Tyr244Ter) - pathogenic g.80881087_80881096dup g.80171370_80171379dup BCKDHB(NM_183050.4):c.722_731dupAAATACTTTA (p.Y244*) - BCKDHB_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 6 c.730T>C r.(?) p.(Tyr244His) - pathogenic (recessive) g.80881095T>C g.80171378T>C 724T>C (Tyr244His) - BCKDHB_000054 - PubMed: Flaschker 2007, Journal: Flaschker 2007 - - Germline - - - - - Johan den Dunnen
-?/. - c.743-8G>T r.(=) p.(=) - likely benign g.80910643G>T g.80200926G>T BCKDHB(NM_000056.3):c.743-8G>T (p.(=)) - BCKDHB_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.743-6A>T r.(=) p.(=) - likely benign g.80910645A>T g.80200928A>T BCKDHB(NM_000056.3):c.743-6A>T (p.(=)) - BCKDHB_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.744G>A r.(?) p.(=) - likely benign g.80910652G>A - BCKDHB(NM_183050.4):c.744G>A (p.(Ala248=)) - BCKDHB_000035 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 7 c.752T>C r.(?) p.(Val251Ala) - pathogenic (recessive) g.80910660T>C g.80200943T>C - - BCKDHB_000055 enzyme activity <0.01 PubMed: Henneke 2003, Journal: Henneke 2003 - - Germline - - - - - Johan den Dunnen
?/. - c.772A>T r.(?) p.(Ile258Phe) - VUS g.80910680A>T - BCKDHB(NM_183050.4):c.772A>T (p.(Ile258Phe)) - BCKDHB_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. - c.799C>T r.(?) p.(Gln267Ter) - pathogenic g.80910707C>T g.80200990C>T BCKDHB(NM_183050.4):c.799C>T (p.Q267*) - BCKDHB_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+?/. 7 c.806G>A r.(?) p.(Gly269Glu) - likely pathogenic (recessive) g.80910714G>A g.80200997G>A - - BCKDHB_000056 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - - Germline - 2/66 alleles MSUD - - - Johan den Dunnen
+/. 7 c.808_821del p.Ser270_Leu274del p.(Asp271CysfsTer25) - pathogenic (recessive) g.80910719_80910732del g.80201002_80201015del - - BCKDHB_000057 - PubMed: Flaschker 2007, Journal: Flaschker 2007 - - Germline - - - - - Johan den Dunnen
?/. - c.832G>A r.(?) p.(Gly278Ser) - VUS g.80910740G>A g.80201023G>A BCKDHB(NM_183050.4):c.832G>A (p.G278S) - BCKDHB_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 7 c.832G>A r.(?) p.(Gly278Ser) - pathogenic (recessive) g.80910740G>A g.80201023G>A - - BCKDHB_000007 - PubMed: Henneke 2003, Journal: Henneke 2003, PubMed: Flaschker 2007 - - Germline - - - - - Johan den Dunnen
+/. 7 c.832G>A r.(?) p.(Gly278Ser) - pathogenic (recessive) g.80910740G>A g.80201023G>A - - BCKDHB_000007 - PubMed: Flaschker 2007, Journal: Flaschker 2007 - - Germline - - - - - Johan den Dunnen
+/. 7 c.832G>A r.(?) p.(Gly278Ser) - pathogenic (recessive) g.80910740G>A g.80201023G>A - - BCKDHB_000007 - PubMed: Flaschker 2007, Journal: Flaschker 2007 - - Germline - - - - - Johan den Dunnen
+/. 7 c.832G>A r.(?) p.(Gly278Ser) - pathogenic (recessive) g.80910740G>A g.80201023G>A - - BCKDHB_000007 - PubMed: Flaschker 2007, Journal: Flaschker 2007 - - Germline - - - - - Johan den Dunnen
-/. - c.841-73C>T r.(?) p.(=) - benign g.80912746C>T g.80203029C>T - - BCKDHB_000058 - Journal: Jaradat 2016 - rs3828753 Germline - - - - - Johan den Dunnen
+/. 8 c.848T>C r.(?) p.(Val283Ala) - pathogenic (recessive) g.80912826T>C g.80203109T>C - - BCKDHB_000059 - PubMed: Flaschker 2007, Journal: Flaschker 2007 - - Germline - - - - - Johan den Dunnen
+/. - c.853C>T r.(?) p.(Arg285Ter) - pathogenic g.80912831C>T g.80203114C>T BCKDHB(NM_183050.4):c.853C>T (p.R285*) - BCKDHB_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. - c.853C>T r.(?) p.(Arg285*) - likely pathogenic g.80912831C>T g.80203114C>T - - BCKDHB_000015 1 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs398124598 Germline - 1/2795 individuals - - - Mohammed Faruq
+/. - c.853C>T r.(?) p.(Arg285Ter) - pathogenic g.80912831C>T - BCKDHB(NM_183050.4):c.853C>T (p.R285*) - BCKDHB_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 8 c.853C>T r.(?) p.(Arg285*) ACMG pathogenic (recessive) g.80912831C>T g.80203114C>T - - BCKDHB_000015 - {PMID:Fang 2021:34556729, Narang 2020:32906206, Mei 2018:30298494} ClinVar-96615 rs398124598 Germline yes - - - - Miriam Erandi Reyna-Fabián
+/. 8 c.853C>T r.(?) p.(Arg285Ter) - pathogenic (recessive) g.80912831C>T g.80203114C>T - - BCKDHB_000015 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - rs398124598 Germline - 2/66 alleles MSUD - - - Johan den Dunnen
+/. 8 c.853C>T r.(?) p.(Arg285Ter) - pathogenic (recessive) g.80912831C>T g.80203114C>T - - BCKDHB_000015 - PubMed: Henneke 2003, Journal: Henneke 2003 - - Germline - - - - - Johan den Dunnen
+/. 8 c.908dup r.(?) p.(Asp303GlufsTer15) - pathogenic (recessive) g.80912886dup g.80203169dup 908_909insA - BCKDHB_000060 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - - Germline - 4/66 alleles MSUD - - - Johan den Dunnen
+/. 8 c.908dup r.(?) p.(Asp303GlufsTer15) - pathogenic (recessive) g.80912886dup g.80203169dup 908_909insA - BCKDHB_000060 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - - Germline - 4/66 alleles MSUD - - - Johan den Dunnen
+/. - c.910del r.(?) p.(Leu304Ter) - pathogenic g.80912888del g.80203171del BCKDHB(NM_183050.4):c.910delC (p.L304*) - BCKDHB_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 8 c.937del r.(?) p.(Asp313ThrfsTer7) - pathogenic (recessive) g.80912915del g.80203198del 937delG - BCKDHB_000061 enzyme activity 0.03 PubMed: Henneke 2003, Journal: Henneke 2003 - - Germline - - - - - Johan den Dunnen
+/. - c.970C>T r.(?) p.(Arg324Ter) - pathogenic g.80982870C>T g.80273153C>T BCKDHB(NM_183050.4):c.970C>T (p.R324*) - BCKDHB_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. - c.970C>T r.(?) p.(Arg324Ter) - pathogenic g.80982870C>T - BCKDHB(NM_183050.4):c.970C>T (p.R324*) - BCKDHB_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. 9 c.970C>T r.(?) p.(Arg324*) ACMG pathogenic (recessive) g.80982870C>T g.80273153C>T - - BCKDHB_000008 Puckett 2010:20307994, Bashyam 2012:22593002, Couce 2015:26232051 - 96621 rs398124603 Germline yes - - - - Miriam Erandi Reyna-Fabián
+/. 9 c.970C>T r.(?) p.(Arg324Ter) - pathogenic (recessive) g.80982870C>T g.80273153C>T - - BCKDHB_000008 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - rs398124603 Germline - 4/66 alleles MSUD - - - Johan den Dunnen
+/. 9 c.970C>T r.(?) p.(Arg324Ter) - pathogenic (recessive) g.80982870C>T g.80273153C>T - - BCKDHB_000008 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - rs398124603 Germline - 4/66 alleles MSUD - - - Johan den Dunnen
+?/. - c.995C>T r.(?) p.(Pro332Leu) - likely pathogenic g.80982895C>T g.80273178C>T - - BCKDHB_000024 - PubMed: Nair 2018 - - Germline/De novo (untested) - - - - - Johan den Dunnen
+?/. 9 c.995C>T r.(?) p.(Pro332Leu) - likely pathogenic (recessive) g.80982895C>T g.80273178C>T - - BCKDHB_000024 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - rs1554205541 Germline - 2/66 alleles MSUD - - - Johan den Dunnen
+?/. - c.1022T>A r.(?) p.(Ile341Asn) - likely pathogenic g.80982922T>A g.80273205T>A - - BCKDHB_000018 4 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs796051939 Germline - 4/2777 individuals - - - Mohammed Faruq
+?/. - c.1038+2T>C r.spl? p.? - likely pathogenic g.80982940T>C - BCKDHB(NM_183050.4):c.1038+2T>C - BCKDHB_000036 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.1046G>A r.(?) p.(Cys349Tyr) - VUS g.81053388G>A g.80343671G>A - - BCKDHB_000019 conflicting interpretations of pathogenicity; 1 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs398124562 Germline - 1/2795 individuals - - - Mohammed Faruq
+/. 10 c.1087T>A r.(?) p.(Tyr363Asn) ACMG likely pathogenic (recessive) g.81053429T>A g.80343712T>A - - BCKDHB_000031 Confirmed by Sanger sequencing. Another patient (with the same phenotype) showed the same variant in heterozygous state together with the pathogenic nonsense variant NM_183050.4:c.564T>A, p.Cys188* - ClinVar-96568 rs398124565 Germline yes 2/97 patients - - - Miriam Erandi Reyna-Fabián
+/. 10 c.1087T>A r.(?) p.(Tyr363Asn) ACMG pathogenic (recessive) g.81053429T>A g.80343712T>A - - BCKDHB_000031 Confirmed by Sanger sequencing. Another patient (with the same phenotype) showed the same variant in homozygous state. - ClinVar-96568 rs398124565 Germline yes 2/97 patients - - - Miriam Erandi Reyna-Fabián
+?/. 10 c.1091A>G r.(?) p.(Asp364Gly) - likely pathogenic (recessive) g.81053433A>G g.80343716A>G - - BCKDHB_000062 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - - Germline - 8/66 alleles MSUD - - - Johan den Dunnen
+?/. 10 c.1091A>G r.(?) p.(Asp364Gly) - likely pathogenic (recessive) g.81053433A>G g.80343716A>G - - BCKDHB_000062 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - - Germline - 8/66 alleles MSUD - - - Johan den Dunnen
+?/. 10 c.1091A>G r.(?) p.(Asp364Gly) - likely pathogenic (recessive) g.81053433A>G g.80343716A>G - - BCKDHB_000062 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - - Germline - 8/66 alleles MSUD - - - Johan den Dunnen
+?/. 10 c.1091A>G r.(?) p.(Asp364Gly) - likely pathogenic (recessive) g.81053433A>G g.80343716A>G - - BCKDHB_000062 - PubMed: Khalifa 2020, Journal: Khalifa 2020 - - Germline - 8/66 alleles MSUD - - - Johan den Dunnen
+/. 10 c.1149T>A r.(?) p.(Tyr383Ter) - pathogenic (recessive) g.81053491T>A g.80343774T>A - - BCKDHB_000063 - PubMed: Henneke 2003, Journal: Henneke 2003 - - Germline - - - - - Johan den Dunnen
+/. 10 c.1149T>A r.(?) p.(Tyr383Ter) - pathogenic (recessive) g.81053491T>A g.80343774T>A - - BCKDHB_000063 enzyme activity <0.01 PubMed: Henneke 2003, Journal: Henneke 2003 - - Germline - - - - - Johan den Dunnen
-/. - c.1150A>G r.(?) p.(Ser384Gly) - benign g.100672060T>C g.100206504T>C - - DBT_000028 12 homozygous controls, 10 heterozygous Journal: Jaradat 2016 - rs12021720 Germline - 34/100 control chromosomes - - - Johan den Dunnen
-/. - c.1221A>G r.(?) p.(Leu407=) - benign g.41930396A>G g.41424491A>G - - BCKDHA_000021 - Journal: Jaradat 2016 - rs4674 Germline - - - - - Johan den Dunnen
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