OGDH — 241 ClinVar variants
All ClinVar germline OGDH variants across every classification, each assembled from ClinVar, gnomAD, AlphaMissense, conservation and more. 100 flagged for review →
4 Pathogenic · 1 Likely pathogenic · 3 Conflicting · 18 Benign · 9 Benign/Likely benign · 98 Likely benign · 108 Uncertain significance
Full OGDH gene report on Sugi Atlas → Gene function, pathways, protein structure, constraint & interactions⚑ Evidence disagreements 100
Variants where the independent computational evidence doesn't line up with the clinical picture — a QC signal for a second look, not a reclassification.
Predictors agree, ClinVar conflicts 3
ClinVar submitters conflict, but the independent predictors unanimously agree — a QC review flag, not a reclassification.
-
OGDH p.Asn820Tyr
Conflicting
Single submitter
oxoglutaricaciduria
-
OGDH p.His3Tyr
Conflicting
Single submitter
oxoglutaricaciduria
-
OGDH p.Val486Met
Conflicting
Single submitter
oxoglutaricaciduria
Predictors lean, ClinVar uncertain 74
ClinVar classifies these uncertain (VUS), but the independent predictors unanimously lean one way — a triage signal for review, not a reclassification.
-
OGDH p.Asn709Ser
Uncertain significance
Multiple submitters
oxoglutaricaciduria
-
OGDH p.Asp811Glu
Uncertain significance
Multiple submitters
oxoglutaricaciduria
-
OGDH p.Lys532Arg
Uncertain significance
Multiple submitters
oxoglutaricaciduria
-
OGDH p.Ser1023Leu
Uncertain significance
Multiple submitters
oxoglutaricaciduria
-
OGDH p.Thr15Met
Uncertain significance
Multiple submitters
oxoglutaricaciduria
-
OGDH p.Thr615Ile
Uncertain significance
Multiple submitters
oxoglutaricaciduria
-
OGDH p.Val808Ile
Uncertain significance
Multiple submitters
oxoglutaricaciduria
- OGDH p.Ala30Thr Uncertain significance Single submitter
-
OGDH p.Ala30Val
Uncertain significance
Single submitter
oxoglutaricaciduria
-
OGDH p.Ala660Val
Uncertain significance
Single submitter
oxoglutaricaciduria
-
OGDH p.Ala815Val
Uncertain significance
Single submitter
oxoglutaricaciduria
-
OGDH p.Ala926Thr
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Arg1008His Uncertain significance Single submitter
- OGDH p.Arg208His Uncertain significance Single submitter
-
OGDH p.Arg252Gln
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Arg270Trp Uncertain significance Single submitter
-
OGDH p.Arg27Ile
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Arg360His Uncertain significance Single submitter
- OGDH p.Arg38Gln Uncertain significance Single submitter
- OGDH p.Arg466Gly Uncertain significance Single submitter
- OGDH p.Arg509Gln Uncertain significance Single submitter
- OGDH p.Arg531His Uncertain significance Single submitter
- OGDH p.Arg642His Uncertain significance Single submitter
- OGDH p.Arg883Trp Uncertain significance Single submitter
- OGDH p.Arg916Cys Uncertain significance Single submitter
- OGDH p.Arg988Gln Uncertain significance Single submitter
-
OGDH p.Asn320Asp
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Asn816Ser Uncertain significance Single submitter
- OGDH p.Asn820Ser Uncertain significance Single submitter
- OGDH p.Asp227His Uncertain significance Single submitter
- OGDH p.Asp363Asn Uncertain significance Single submitter
- OGDH p.Asp873Glu Uncertain significance Single submitter
- OGDH p.Cys395Arg Uncertain significance Single submitter
- OGDH p.Cys904Ser Uncertain significance Single submitter
- OGDH p.Gln598Lys Uncertain significance Single submitter
- OGDH p.Gln800Glu Uncertain significance Single submitter
-
OGDH p.Glu266Asp
Uncertain significance
Single submitter
oxoglutaricaciduria
-
OGDH p.Glu481Lys
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Gly173Glu Uncertain significance Single submitter
-
OGDH p.Gly548Ala
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Gly643Arg Uncertain significance Single submitter
- OGDH p.Gly86Val Uncertain significance Single submitter
-
OGDH p.His109Tyr
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.His3Arg Uncertain significance Single submitter
- OGDH p.Ile134Asn Uncertain significance Single submitter
- OGDH p.Ile207Phe Uncertain significance Single submitter
-
OGDH p.Ile294Thr
Uncertain significance
Single submitter
oxoglutaricaciduria
-
OGDH p.Ile613Val
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Ile977Met Uncertain significance Single submitter
-
OGDH p.Leu609Val
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Lys10Arg Uncertain significance Single submitter
- OGDH p.Lys981Arg Uncertain significance Single submitter
- OGDH p.Met61Ile Uncertain significance Single submitter
-
OGDH p.Met921Val
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Pro241Leu Uncertain significance Single submitter
- OGDH p.Pro605Ser Uncertain significance Single submitter
- OGDH p.Pro886Ala Uncertain significance Single submitter
- OGDH p.Ser24Leu Uncertain significance Single submitter
- OGDH p.Ser54Asn Uncertain significance Single submitter
-
OGDH p.Ser606Cys
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Ser792Ala Uncertain significance Single submitter
- OGDH p.Thr1004Met Uncertain significance Single submitter
-
OGDH p.Thr169Ala
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Thr913Asn Uncertain significance Single submitter
- OGDH p.Thr976Ile Uncertain significance Single submitter
- OGDH p.Tyr175His Uncertain significance Single submitter
- OGDH p.Tyr432His Uncertain significance Single submitter
- OGDH p.Val107Met Uncertain significance Single submitter
-
OGDH p.Val120Met
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Val438Met Uncertain significance Single submitter
- OGDH p.Val44Phe Uncertain significance Single submitter
- OGDH p.Val715Leu Uncertain significance Single submitter
-
OGDH p.Val945Ala
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Ala213Thr Uncertain significance No assertion criteria
Predictors split 23
The independent predictors disagree with each other — read with care.
-
OGDH p.Arg204Trp
Uncertain significance
Multiple submitters
oxoglutaricaciduria
-
OGDH p.Ser55Leu
Benign/Likely benign
Multiple submitters
oxoglutaricaciduria
-
OGDH p.Thr610Met
Uncertain significance
Multiple submitters
oxoglutaricaciduria
- OGDH p.Ala733Thr Uncertain significance Single submitter
-
OGDH p.Arg208Cys
Uncertain significance
Single submitter
oxoglutaricaciduria
-
OGDH p.Arg257Lys
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Arg260Gln Uncertain significance Single submitter
- OGDH p.Arg531Cys Uncertain significance Single submitter
- OGDH p.Asn56Lys Uncertain significance Single submitter
-
OGDH p.Asn950Ser
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Glu391Lys Uncertain significance Single submitter
- OGDH p.Glu915Lys Uncertain significance Single submitter
- OGDH p.Leu901Phe Uncertain significance Single submitter
- OGDH p.Lys184Asn Uncertain significance Single submitter
-
OGDH p.Pro162Leu
Uncertain significance
Single submitter
oxoglutaricaciduria
- OGDH p.Pro764Leu Uncertain significance Single submitter
- OGDH p.Pro866Arg Uncertain significance Single submitter
- OGDH p.Pro890Arg Uncertain significance Single submitter
-
OGDH p.Pro98Leu
Uncertain significance
Single submitter
oxoglutaricaciduria
-
OGDH p.Ser495Arg
Uncertain significance
Single submitter
oxoglutaricaciduria
-
OGDH p.Val302Leu
Uncertain significance
Single submitter
oxoglutaricaciduria
-
OGDH p.Val305Met
Likely benign
Single submitter
oxoglutaricaciduria
-
OGDH p.Val908Met
Uncertain significance
Single submitter
oxoglutaricaciduria
All OGDH variants
Pathogenic 4
- p.Arg312Lys No assertion criteria oxoglutaricaciduria
- p.Asn320Ser No assertion criteria oxoglutaricaciduria
- p.Pro189Leu No assertion criteria oxoglutaricaciduria
- p.Ser297Tyr No assertion criteria oxoglutaricaciduria
Likely pathogenic 1
- c.1669-9A>G Single submitter oxoglutaricaciduria
Conflicting classifications of pathogenicity 3
- p.Asn820Tyr Single submitter oxoglutaricaciduria ⚠
- p.His3Tyr Single submitter oxoglutaricaciduria ⚠
- p.Val486Met Single submitter oxoglutaricaciduria ⚠
Benign 18
- p.Asn619= Multiple submitters oxoglutaricaciduria
- c.222+9C>T Single submitter oxoglutaricaciduria
- c.2632+17T>C Single submitter oxoglutaricaciduria
- c.414+20C>T Single submitter oxoglutaricaciduria
- c.518-15C>T Single submitter oxoglutaricaciduria
- c.935+15G>A Single submitter oxoglutaricaciduria
- p.Ala991= Single submitter oxoglutaricaciduria
- p.Asn1021= Single submitter oxoglutaricaciduria
- p.Asn119= Single submitter oxoglutaricaciduria
- p.Asp1017= Single submitter oxoglutaricaciduria
- p.Glu115= Single submitter oxoglutaricaciduria
- p.His474= Single submitter oxoglutaricaciduria
- p.Ser132= Single submitter oxoglutaricaciduria
- p.Ser344= Single submitter oxoglutaricaciduria
- p.Thr928= Single submitter oxoglutaricaciduria
- p.Thr996= Single submitter oxoglutaricaciduria
- p.Val1018Ile Single submitter oxoglutaricaciduria
- p.Val715= Single submitter oxoglutaricaciduria
Benign/Likely benign 9
- c.1206+19C>A Multiple submitters oxoglutaricaciduria
- c.936-7G>A Multiple submitters oxoglutaricaciduria
- p.Ala379= Multiple submitters oxoglutaricaciduria
- p.Ala656= Multiple submitters oxoglutaricaciduria
- p.Gly396= Multiple submitters oxoglutaricaciduria
- p.Phe1014= Multiple submitters oxoglutaricaciduria
- p.Ser55Leu Multiple submitters oxoglutaricaciduria ⚠
- p.Thr610= Multiple submitters oxoglutaricaciduria
- p.Val384= Multiple submitters oxoglutaricaciduria
Likely benign 98
- p.Asn316= Multiple submitters
- p.Tyr968= Multiple submitters oxoglutaricaciduria
- c.1027-9C>T Single submitter oxoglutaricaciduria
- c.1335+19C>T Single submitter oxoglutaricaciduria
- c.1516-4G>A Single submitter
- c.1668+13G>A Single submitter oxoglutaricaciduria
- c.1771+16G>A Single submitter oxoglutaricaciduria
- c.1772-13C>G Single submitter oxoglutaricaciduria
- c.1772-16G>A Single submitter oxoglutaricaciduria
- c.1900+7C>T Single submitter oxoglutaricaciduria
- c.1900+8G>A Single submitter oxoglutaricaciduria
- c.1901-8C>G Single submitter oxoglutaricaciduria
- c.2051+10T>C Single submitter oxoglutaricaciduria
- c.2051+12G>T Single submitter oxoglutaricaciduria
- c.2179+17A>G Single submitter
- c.2180-8C>T Single submitter oxoglutaricaciduria
- c.2431-15C>A Single submitter oxoglutaricaciduria
- c.2632+16A>T Single submitter oxoglutaricaciduria
- c.2632+7G>T Single submitter oxoglutaricaciduria
- c.2633-8T>C Single submitter oxoglutaricaciduria
- c.2797-10T>C Single submitter oxoglutaricaciduria
- c.2952-20C>T Single submitter oxoglutaricaciduria
- c.2952-9A>G Single submitter
- c.414+13C>T Single submitter oxoglutaricaciduria
- c.415-14T>C Single submitter oxoglutaricaciduria
- c.517+10T>C Single submitter oxoglutaricaciduria
- c.518-14A>C Single submitter oxoglutaricaciduria
- c.633+16G>C Single submitter oxoglutaricaciduria
- c.935+17G>A Single submitter oxoglutaricaciduria
- c.936-16C>T Single submitter oxoglutaricaciduria
- p.Ala372= Single submitter oxoglutaricaciduria
- p.Ala490= Single submitter oxoglutaricaciduria
- p.Ala491= Single submitter oxoglutaricaciduria
- p.Ala85= Single submitter oxoglutaricaciduria
- p.Ala986= Single submitter oxoglutaricaciduria
- p.Arg135= Single submitter oxoglutaricaciduria
- p.Arg38= Single submitter oxoglutaricaciduria
- p.Arg883= Single submitter
- p.Arg914= Single submitter oxoglutaricaciduria
- p.Asn511= Single submitter oxoglutaricaciduria
- p.Asn514= Single submitter oxoglutaricaciduria
- p.Asp158= Single submitter oxoglutaricaciduria
- p.Asp596= Single submitter oxoglutaricaciduria
- p.Asp677= Single submitter oxoglutaricaciduria
- p.Cys283= Single submitter
- p.Gly90= Single submitter oxoglutaricaciduria
- p.His109= Single submitter oxoglutaricaciduria
- p.His376= Single submitter oxoglutaricaciduria
- p.His439= Single submitter oxoglutaricaciduria
- p.His840= Single submitter oxoglutaricaciduria
- p.Ile37= Single submitter
- p.Ile856Val Single submitter
- p.Ile927Val Single submitter oxoglutaricaciduria
- p.Leu113= Single submitter oxoglutaricaciduria
- p.Leu177= Single submitter oxoglutaricaciduria
- p.Leu280= Single submitter oxoglutaricaciduria
- p.Leu337= Single submitter
- p.Leu407= Single submitter
- p.Leu595= Single submitter oxoglutaricaciduria
- p.Leu664= Single submitter oxoglutaricaciduria
- p.Leu665= Single submitter oxoglutaricaciduria
- p.Leu742= Single submitter oxoglutaricaciduria
- p.Leu799= Single submitter oxoglutaricaciduria
- p.Leu842= Single submitter oxoglutaricaciduria
- p.Lys999= Single submitter oxoglutaricaciduria
- p.Phe761= Single submitter oxoglutaricaciduria
- p.Phe817= Single submitter oxoglutaricaciduria
- p.Pro13= Single submitter oxoglutaricaciduria
- p.Pro382= Single submitter oxoglutaricaciduria
- p.Pro480= Single submitter oxoglutaricaciduria
- p.Pro553= Single submitter oxoglutaricaciduria
- p.Pro89= Single submitter oxoglutaricaciduria
- p.Pro937= Single submitter oxoglutaricaciduria
- p.Pro98= Single submitter oxoglutaricaciduria
- p.Ser546= Single submitter oxoglutaricaciduria
- p.Ser601= Single submitter oxoglutaricaciduria
- p.Ser792= Single submitter oxoglutaricaciduria
- p.Ser934= Single submitter
- p.Thr1001= Single submitter oxoglutaricaciduria
- p.Thr1004= Single submitter oxoglutaricaciduria
- p.Thr1012= Single submitter oxoglutaricaciduria
- p.Thr15= Single submitter oxoglutaricaciduria
- p.Thr367= Single submitter oxoglutaricaciduria
- p.Thr522= Single submitter
- p.Thr6= Single submitter oxoglutaricaciduria
- p.Thr83= Single submitter oxoglutaricaciduria
- p.Tyr658= Single submitter oxoglutaricaciduria
- p.Tyr909= Single submitter oxoglutaricaciduria
- p.Val305= Single submitter oxoglutaricaciduria
- p.Val305Met Single submitter oxoglutaricaciduria ⚠
- p.Val441= Single submitter
- p.Val626= Single submitter oxoglutaricaciduria
- p.Val898= Single submitter
- c.2051+9del No assertion criteria OGDH-related disorder
- c.2559+4A>G No assertion criteria OGDH-related disorder
- c.936-8C>T No assertion criteria OGDH-related disorder
- p.Ala660= No assertion criteria OGDH-related disorder
- p.Tyr57= No assertion criteria OGDH-related disorder
Uncertain significance 108
- p.Arg204Trp Multiple submitters oxoglutaricaciduria ⚠
- p.Asn709Ser Multiple submitters oxoglutaricaciduria ⚠
- p.Asp811Glu Multiple submitters oxoglutaricaciduria ⚠
- p.Lys532Arg Multiple submitters oxoglutaricaciduria ⚠
- p.Ser1023Leu Multiple submitters oxoglutaricaciduria ⚠
- p.Thr15Met Multiple submitters oxoglutaricaciduria ⚠
- p.Thr610Met Multiple submitters oxoglutaricaciduria ⚠
- p.Thr615Ile Multiple submitters oxoglutaricaciduria ⚠
- p.Val646Leu Multiple submitters oxoglutaricaciduria
- p.Val808Ile Multiple submitters oxoglutaricaciduria ⚠
- c.1026+6G>A Single submitter oxoglutaricaciduria
- c.1901-3C>T Single submitter oxoglutaricaciduria
- c.2051+6G>A Single submitter oxoglutaricaciduria
- c.2052-3C>T Single submitter oxoglutaricaciduria
- c.2358+6C>T Single submitter oxoglutaricaciduria
- c.2951+4A>G Single submitter oxoglutaricaciduria
- c.936-17T>G Single submitter oxoglutaricaciduria
- p.Ala30Thr Single submitter ⚠
- p.Ala30Val Single submitter oxoglutaricaciduria ⚠
- p.Ala660Val Single submitter oxoglutaricaciduria ⚠
- p.Ala733Thr Single submitter ⚠
- p.Ala815Val Single submitter oxoglutaricaciduria ⚠
- p.Ala926Thr Single submitter oxoglutaricaciduria ⚠
- p.Arg1008His Single submitter ⚠
- p.Arg208Cys Single submitter oxoglutaricaciduria ⚠
- p.Arg208His Single submitter ⚠
- p.Arg252Gln Single submitter oxoglutaricaciduria ⚠
- p.Arg257Lys Single submitter oxoglutaricaciduria ⚠
- p.Arg260Gln Single submitter ⚠
- p.Arg270Trp Single submitter ⚠
- p.Arg27Ile Single submitter oxoglutaricaciduria ⚠
- p.Arg360His Single submitter ⚠
- p.Arg38Gln Single submitter ⚠
- p.Arg466Gly Single submitter ⚠
- p.Arg509Gln Single submitter ⚠
- p.Arg531Cys Single submitter ⚠
- p.Arg531His Single submitter ⚠
- p.Arg642His Single submitter ⚠
- p.Arg883Trp Single submitter ⚠
- p.Arg916Cys Single submitter ⚠
- p.Arg988Gln Single submitter ⚠
- p.Asn320Asp Single submitter oxoglutaricaciduria ⚠
- p.Asn56Lys Single submitter ⚠
- p.Asn816Ser Single submitter ⚠
- p.Asn820Ser Single submitter ⚠
- p.Asn950Ser Single submitter oxoglutaricaciduria ⚠
- p.Asp227His Single submitter ⚠
- p.Asp363Asn Single submitter ⚠
- p.Asp824Val Single submitter
- p.Asp873Glu Single submitter ⚠
- p.Cys395Arg Single submitter ⚠
- p.Cys904Ser Single submitter ⚠
- p.Gln269_Arg270delinsTer Single submitter
- p.Gln598Lys Single submitter ⚠
- p.Gln800Glu Single submitter ⚠
- p.Glu266Asp Single submitter oxoglutaricaciduria ⚠
- p.Glu391Lys Single submitter ⚠
- p.Glu481Lys Single submitter oxoglutaricaciduria ⚠
- p.Glu492Ter Single submitter oxoglutaricaciduria
- p.Glu915Lys Single submitter ⚠
- p.Gly173Glu Single submitter ⚠
- p.Gly548Ala Single submitter oxoglutaricaciduria ⚠
- p.Gly643Arg Single submitter ⚠
- p.Gly86Val Single submitter ⚠
- p.His109Tyr Single submitter oxoglutaricaciduria ⚠
- p.His3Arg Single submitter ⚠
- p.Ile134Asn Single submitter ⚠
- p.Ile207Phe Single submitter ⚠
- p.Ile294Thr Single submitter oxoglutaricaciduria ⚠
- p.Ile613Val Single submitter oxoglutaricaciduria ⚠
- p.Ile977Met Single submitter ⚠
- p.Leu609Val Single submitter oxoglutaricaciduria ⚠
- p.Leu901Phe Single submitter ⚠
- p.Lys1020fs Single submitter oxoglutaricaciduria
- p.Lys10Arg Single submitter ⚠
- p.Lys184Asn Single submitter ⚠
- p.Lys981Arg Single submitter ⚠
- p.Met61Ile Single submitter ⚠
- p.Met921Val Single submitter oxoglutaricaciduria ⚠
- p.Pro162Leu Single submitter oxoglutaricaciduria ⚠
- p.Pro241Leu Single submitter ⚠
- p.Pro605Ser Single submitter ⚠
- p.Pro764Leu Single submitter ⚠
- p.Pro866Arg Single submitter ⚠
- p.Pro886Ala Single submitter ⚠
- p.Pro890Arg Single submitter ⚠
- p.Pro98Leu Single submitter oxoglutaricaciduria ⚠
- p.Ser24Leu Single submitter ⚠
- p.Ser495Arg Single submitter oxoglutaricaciduria ⚠
- p.Ser54Asn Single submitter ⚠
- p.Ser606Cys Single submitter oxoglutaricaciduria ⚠
- p.Ser792Ala Single submitter ⚠
- p.Thr1004Met Single submitter ⚠
- p.Thr169Ala Single submitter oxoglutaricaciduria ⚠
- p.Thr753Met Single submitter
- p.Thr913Asn Single submitter ⚠
- p.Thr976Ile Single submitter ⚠
- p.Tyr175His Single submitter ⚠
- p.Tyr432His Single submitter ⚠
- p.Val107Met Single submitter ⚠
- p.Val120Met Single submitter oxoglutaricaciduria ⚠
- p.Val302Leu Single submitter oxoglutaricaciduria ⚠
- p.Val438Met Single submitter ⚠
- p.Val44Phe Single submitter ⚠
- p.Val715Leu Single submitter ⚠
- p.Val908Met Single submitter oxoglutaricaciduria ⚠
- p.Val945Ala Single submitter oxoglutaricaciduria ⚠
- p.Ala213Thr No assertion criteria ⚠