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Items: 40

Variation
Gene
(Protein Change)
Type
(Consequence)
ConditionClassification, Review status
BCKDHA
(M1I)
Single nucleotide variant
(missense variant +1 more)
Maple syrup urine disease
GLikely pathogenic
BCKDHA
Deletion
Maple syrup urine disease
GUncertain significance
BCKDHA
(R40fs)
Duplication
(frameshift variant)
Maple syrup urine disease
+3 more
GPathogenic
BCKDHA
(R40fs)
Deletion
(frameshift variant)
Maple syrup urine disease
+2 more
GPathogenic
BCKDHA
Microsatellite
(inframe_insertion)
Maple syrup urine disease
GUncertain significance
BCKDHA
(Q43*)
Single nucleotide variant
(nonsense)
Maple syrup urine disease
GPathogenic/Likely pathogenic
BCKDHA
(S46*)
Single nucleotide variant
(nonsense)
Maple syrup urine disease type 1A
+1 more
GPathogenic/Likely pathogenic
BCKDHA
(R159W)
Single nucleotide variant
(missense variant)
Maple syrup urine disease
GConflicting classifications of pathogenicity
BCKDHA
(R167Q)
Single nucleotide variant
(missense variant)
Maple syrup urine disease
GUncertain significance
BCKDHA
(G188W)
Single nucleotide variant
(missense variant)
Maple syrup urine disease
GUncertain significance
BCKDHA
(T211M)
Single nucleotide variant
(missense variant)
Maple syrup urine disease type 1A
+2 more
GPathogenic/Likely pathogenic
BCKDHA
(A220V)
Single nucleotide variant
(missense variant)
not provided
+1 more
GPathogenic/Likely pathogenic
BCKDHA
(A240fs)
Deletion
(frameshift variant)
Maple syrup urine disease type 1A
+1 more
GPathogenic
BCKDHA
(A253T)
Single nucleotide variant
(missense variant)
Maple syrup urine disease
GPathogenic/Likely pathogenic
BCKDHA
(R265P)
Single nucleotide variant
(missense variant)
Maple syrup urine disease
GConflicting classifications of pathogenicity
BCKDHA
(A285P)
Single nucleotide variant
(missense variant)
not provided
+2 more
GPathogenic/Likely pathogenic
BCKDHA
Single nucleotide variant
(splice donor variant)
Maple syrup urine disease
GLikely pathogenic
BCKDHA
(R287* +1 more)
Single nucleotide variant
(nonsense)
Maple syrup urine disease
+1 more
GPathogenic
BCKDHA
(G288fs +1 more)
Deletion
(frameshift variant)
not provided
+2 more
GPathogenic
BCKDHA
(G289R +1 more)
Single nucleotide variant
(missense variant)
not provided
+2 more
GPathogenic/Likely pathogenic
BCKDHA
(R297C +1 more)
Single nucleotide variant
(missense variant)
Maple syrup urine disease type 1A
+2 more
GConflicting classifications of pathogenicity
BCKDHA
(R297H +1 more)
Single nucleotide variant
(missense variant)
not provided
+2 more
GConflicting classifications of pathogenicity
BCKDHA
(V298M +1 more)
Single nucleotide variant
(missense variant)
not specified
+1 more
GUncertain significance
BCKDHA
(R314* +1 more)
Single nucleotide variant
(nonsense)
Maple syrup urine disease
+1 more
GPathogenic
BCKDHA
(E327K +1 more)
Single nucleotide variant
(missense variant)
not provided
+2 more
GPathogenic/Likely pathogenic
BCKDHA
(Y330* +1 more)
Duplication
(nonsense)
Maple syrup urine disease
GLikely pathogenic
BCKDHA
(H335fs +1 more)
Deletion
(frameshift variant)
not provided
+2 more
GPathogenic/Likely pathogenic
BCKDHA
(R346C +1 more)
Single nucleotide variant
(missense variant)
Maple syrup urine disease
+3 more
GConflicting classifications of pathogenicity
BCKDHA
(R346H +1 more)
Single nucleotide variant
(missense variant)
not provided
+2 more
GPathogenic/Likely pathogenic
BCKDHA
(R363W +1 more)
Single nucleotide variant
(missense variant)
Maple syrup urine disease
+1 more
GPathogenic/Likely pathogenic
BCKDHA
(W373* +1 more)
Single nucleotide variant
(nonsense)
Maple syrup urine disease
GPathogenic/Likely pathogenic
BCKDHA
Deletion
(splice donor variant)
Maple syrup urine disease
GPathogenic/Likely pathogenic
BCKDHA
Single nucleotide variant
(splice acceptor variant)
not provided
+2 more
GPathogenic/Likely pathogenic
BCKDHA
(E398* +1 more)
Single nucleotide variant
(nonsense)
Maple syrup urine disease
GLikely pathogenic
BCKDHA
(K400* +1 more)
Single nucleotide variant
(nonsense)
Maple syrup urine disease
GPathogenic/Likely pathogenic
BCKDHA
(V412M +1 more)
Single nucleotide variant
(missense variant)
not provided
+3 more
GPathogenic/Likely pathogenic
BCKDHA
(Q424del +1 more)
Microsatellite
(inframe_deletion)
Maple syrup urine disease
GUncertain significance
BCKDHA
Deletion
(inframe_indel)
Maple syrup urine disease type 1A
+1 more
GPathogenic/Likely pathogenic
BCKDHA
(Y438N +1 more)
Single nucleotide variant
(missense variant)
not provided
+3 more
GPathogenic/Likely pathogenic
BCKDHA
Single nucleotide variant
(3 prime UTR variant)
Maple syrup urine disease
GUncertain significance
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