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Items: 41

  • The following term was not found in ClinVar: dinitrobenzenesulfonic.
Variation
Gene
(Protein Change)
Type
(Consequence)
ConditionClassification, Review status
HSD3B2
(A204D)
Single nucleotide variant
(missense variant)
3 beta-Hydroxysteroid dehydrogenase deficiency
GUncertain significance
HMGCS2
(N276S +1 more)
Single nucleotide variant
(missense variant)
HMGCS2-related disorder
+2 more
GUncertain significance
HMGCS2
(T260I +1 more)
Single nucleotide variant
(missense variant)
3-hydroxy-3-methylglutaryl-CoA synthase deficiency
GLikely pathogenic
HMGCS2
(M256I +1 more)
Single nucleotide variant
(missense variant)
3-hydroxy-3-methylglutaryl-CoA synthase deficiency
+1 more
GUncertain significance
HMGCS2
(A284G +1 more)
Single nucleotide variant
(missense variant)
3-hydroxy-3-methylglutaryl-CoA synthase deficiency
+1 more
GUncertain significance
HMGCS2
(R232H +1 more)
Single nucleotide variant
(missense variant)
not specified
+1 more
GConflicting classifications of pathogenicity
HMGCS2
(G55D)
Single nucleotide variant
(missense variant)
3-hydroxy-3-methylglutaryl-CoA synthase deficiency
GLikely pathogenic
HADHA
Single nucleotide variant
(intron variant)
not provided
+2 more
GPathogenic/Likely pathogenic
RBSN
(G425R)
Single nucleotide variant
(missense variant)
not provided
GConflicting classifications of pathogenicity
HMGCS1
(M219V +1 more)
Single nucleotide variant
(missense variant)
not specified
GUncertain significance
HMGCR
(L97H)
Single nucleotide variant
(missense variant)
Inborn genetic diseases
GUncertain significance
HMGCR
(R136K)
Single nucleotide variant
(missense variant)
Inborn genetic diseases
GUncertain significance
HMGCR
(G769D +1 more)
Single nucleotide variant
(missense variant)
Limb-girdle muscular dystrophy
GPathogenic
CYP2C9
(R144C)
Single nucleotide variant
(missense variant)
Lesinurad response
+4 more
GLikely benign; drug response; other
HOGA1
Single nucleotide variant
(intron variant)
Primary hyperoxaluria type 3
+1 more
GPathogenic
HOGA1
(G287V +1 more)
Single nucleotide variant
(missense variant)
Primary hyperoxaluria type 3
+1 more
GPathogenic/Likely pathogenic
HSD3B7
(G17fs)
Deletion
(frameshift variant)
not provided
+1 more
GPathogenic
HSD3B7
(P48R)
Single nucleotide variant
(missense variant)
Congenital bile acid synthesis defect 1
GUncertain significance
HSD3B7
(Q69*)
Single nucleotide variant
(nonsense)
Congenital bile acid synthesis defect 1
GLikely pathogenic
HSD3B7
(K99fs)
Duplication
(frameshift variant)
not provided
GPathogenic
HSD3B7
Single nucleotide variant
(splice donor variant)
Congenital bile acid synthesis defect 1
GPathogenic
HSD3B7
Single nucleotide variant
(splice donor variant)
Congenital bile acid synthesis defect 1
GLikely pathogenic
HSD3B7
(E147K)
Single nucleotide variant
(missense variant)
not provided
+1 more
GPathogenic
HSD3B7
(E152K)
Single nucleotide variant
(missense variant)
Inborn genetic diseases
+1 more
GUncertain significance
HSD3B7
(E167*)
Single nucleotide variant
(nonsense)
Congenital bile acid synthesis defect 1
+1 more
GPathogenic
HSD3B7
(T186M)
Single nucleotide variant
(missense variant +1 more)
Congenital bile acid synthesis defect 1
GUncertain significance
HSD3B7
Single nucleotide variant
(synonymous variant +1 more)
Congenital bile acid synthesis defect 1
+1 more
GConflicting classifications of pathogenicity
HSD3B7
(R190H)
Single nucleotide variant
(missense variant +1 more)
Congenital bile acid synthesis defect 1
GUncertain significance
HSD3B7
(G196S)
Single nucleotide variant
(missense variant +1 more)
Congenital bile acid synthesis defect 1
+1 more
GUncertain significance
HSD3B7
(R203K)
Single nucleotide variant
(missense variant +1 more)
not provided
+1 more
GUncertain significance
HSD3B7
(G213R)
Single nucleotide variant
(missense variant +1 more)
Inborn genetic diseases
+1 more
GUncertain significance
HSD3B7
(V224A)
Single nucleotide variant
(missense variant +1 more)
Inborn genetic diseases
+1 more
GUncertain significance
HSD3B7
(R228W)
Single nucleotide variant
(missense variant +1 more)
Congenital bile acid synthesis defect 1
+1 more
GConflicting classifications of pathogenicity
HSD3B7
(Y230C)
Single nucleotide variant
(missense variant +1 more)
Congenital bile acid synthesis defect 1
GUncertain significance
HSD3B7
Deletion
(intron variant +1 more)
Congenital bile acid synthesis defect 1
GPathogenic
HSD3B7
(T250A)
Single nucleotide variant
(synonymous variant +1 more)
not specified
+2 more
GBenign
HSD3B7
(T323M)
Single nucleotide variant
(3 prime UTR variant +1 more)
Congenital bile acid synthesis defect 1
GUncertain significance
HSD3B7
(Y344C)
Single nucleotide variant
(3 prime UTR variant +1 more)
Congenital bile acid synthesis defect 1
GUncertain significance
HSD3B7
(L347fs)
Deletion
(3 prime UTR variant +1 more)
Congenital bile acid synthesis defect 1
GPathogenic
HSD3B7
Single nucleotide variant
(3 prime UTR variant +1 more)
not specified
+2 more
GBenign
SGCA
(I124T)
Single nucleotide variant
(missense variant +1 more)
not provided
+1 more
GPathogenic/Likely pathogenic
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