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Items: 69

Variation
Gene
(Protein Change)
Type
(Consequence)
ConditionClassification, Review status
NDUFS2
(Y53C)
Single nucleotide variant
(missense variant +1 more)
Mitochondrial complex 1 deficiency, nuclear type 6
+3 more
GUncertain significance
NDUFS2
Deletion
(intron variant)
not provided
+1 more
GConflicting classifications of pathogenicity
NDUFS2
Single nucleotide variant
(3 prime UTR variant +2 more)
Mitochondrial complex I deficiency, nuclear type 1
+1 more
GConflicting classifications of pathogenicity
NDUFS1
Single nucleotide variant
(intron variant)
Mitochondrial complex I deficiency, nuclear type 1
GUncertain significance
NDUFS1
(N669S +4 more)
Single nucleotide variant
(missense variant)
Leigh syndrome
+3 more
GUncertain significance
NDUFS1
(P506S +4 more)
Single nucleotide variant
(missense variant)
Inborn genetic diseases
+2 more
GUncertain significance
NDUFS1
(V506I +4 more)
Single nucleotide variant
(missense variant)
not provided
+4 more
GConflicting classifications of pathogenicity
NDUFS1
(V343del +4 more)
Microsatellite
(inframe_deletion)
Mitochondrial complex I deficiency, nuclear type 1
GUncertain significance
NDUFS1
(R219C +4 more)
Single nucleotide variant
(missense variant)
not provided
+2 more
GUncertain significance
NDUFS1
(A173V +4 more)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency, nuclear type 1
+1 more
GUncertain significance
NDUFS1
(M37V +3 more)
Single nucleotide variant
(missense variant +1 more)
Mitochondrial complex I deficiency, nuclear type 1
+1 more
GUncertain significance
LOC129936730, NDUFAF3
(A37T)
Single nucleotide variant
(missense variant +1 more)
Mitochondrial complex I deficiency, nuclear type 1
+1 more
GUncertain significance
NDUFAF3
(Y63* +1 more)
Duplication
(nonsense)
not provided
+3 more
GConflicting classifications of pathogenicity
NDUFAF3
(Q129R +1 more)
Single nucleotide variant
(missense variant)
not provided
+2 more
GUncertain significance
TIMMDC1
(R59W)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency, nuclear type 1
GUncertain significance
TIMMDC1
(I193T)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency, nuclear type 1
+2 more
GUncertain significance
TIMMDC1
(R225Q)
Single nucleotide variant
(missense variant)
not provided
+1 more
GUncertain significance
TIMMDC1
(K249E)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency, nuclear type 1
+1 more
GConflicting classifications of pathogenicity
LOC129993885, NDUFS4
(M1I)
Single nucleotide variant
(missense variant +2 more)
Mitochondrial complex I deficiency, nuclear type 1
GUncertain significance
LOC129993885, NDUFS4
(S7*)
Single nucleotide variant
(nonsense +1 more)
Mitochondrial complex I deficiency, nuclear type 1
GLikely pathogenic
LOC129993885, NDUFS4
(V9fs)
Deletion
(frameshift variant +1 more)
Mitochondrial complex I deficiency, nuclear type 1
+1 more
GPathogenic/Likely pathogenic
NDUFS4
Single nucleotide variant
(splice acceptor variant)
not provided
+2 more
GPathogenic
NDUFS4
(T39fs)
Deletion
(frameshift variant +1 more)
Mitochondrial complex I deficiency, nuclear type 1
GLikely pathogenic
NDUFS4
(Q46fs)
Duplication
(frameshift variant +1 more)
Mitochondrial complex I deficiency, nuclear type 1
GLikely pathogenic
NDUFS4
(T74fs)
Deletion
(frameshift variant +1 more)
Leigh syndrome
+1 more
GPathogenic/Likely pathogenic
NDUFS4
(I79fs)
Deletion
(frameshift variant +1 more)
Mitochondrial complex I deficiency, nuclear type 1
GLikely pathogenic
NDUFS4
Deletion
(nonsense +1 more)
Mitochondrial complex I deficiency, nuclear type 1
+2 more
GPathogenic
NDUFS4
(M99fs)
Deletion
(frameshift variant +1 more)
Mitochondrial complex I deficiency, nuclear type 1
GLikely pathogenic
NDUFS4
(R106*)
Single nucleotide variant
(nonsense +1 more)
Mitochondrial complex I deficiency, nuclear type 1
+3 more
GPathogenic
NDUFS4
(W107fs)
Deletion
(frameshift variant +1 more)
Inborn genetic diseases
+1 more
GPathogenic/Likely pathogenic
NDUFS4
(W114*)
Single nucleotide variant
(nonsense +1 more)
Mitochondrial complex I deficiency, nuclear type 1
GLikely pathogenic
NDUFS4
Single nucleotide variant
(splice donor variant)
not provided
+2 more
GPathogenic/Likely pathogenic
NDUFS4
Single nucleotide variant
(splice acceptor variant +1 more)
Mitochondrial complex I deficiency, nuclear type 1
GLikely pathogenic
NDUFS4
(D119H)
Single nucleotide variant
(missense variant +2 more)
Mitochondrial complex I deficiency
+3 more
GLikely pathogenic
NDUFS4
(E132fs)
Duplication
(frameshift variant +2 more)
not provided
+1 more
GPathogenic
NDUFS4
(E139*)
Single nucleotide variant
(nonsense +2 more)
Mitochondrial complex I deficiency, nuclear type 1
GLikely pathogenic
NDUFS4
(G142*)
Single nucleotide variant
(nonsense +2 more)
Mitochondrial complex I deficiency, nuclear type 1
GLikely pathogenic
NDUFS4
Single nucleotide variant
(splice donor variant +1 more)
Mitochondrial complex I deficiency, nuclear type 1
GLikely pathogenic
NDUFS4
(K154fs)
Deletion
(3 prime UTR variant +2 more)
Leigh syndrome
+3 more
GPathogenic
NDUFS4
(K158fs)
Duplication
(3 prime UTR variant +2 more)
Mitochondrial complex I deficiency, nuclear type 1
+2 more
GPathogenic/Likely pathogenic
NDUFS4
(Y160fs)
Duplication
(3 prime UTR variant +2 more)
Mitochondrial complex I deficiency, nuclear type 1
+1 more
GLikely pathogenic
NDUFS4
(Y160*)
Duplication
(3 prime UTR variant +2 more)
Mitochondrial complex I deficiency, nuclear type 1
GLikely pathogenic
NDUFS4
(R169T)
Single nucleotide variant
(3 prime UTR variant +2 more)
Mitochondrial complex I deficiency, nuclear type 1
GUncertain significance
ERCC8, NDUFAF2
(Y38*)
Single nucleotide variant
(nonsense)
Mitochondrial complex 1 deficiency, nuclear type 10
+3 more
GPathogenic
NDUFAF2
(R47*)
Single nucleotide variant
(nonsense)
Mitochondrial complex I deficiency, nuclear type 1
+4 more
GPathogenic/Likely pathogenic
NDUFAF4
(P90S)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency, nuclear type 1
GUncertain significance
NDUFV1
(R40Q +1 more)
Single nucleotide variant
(missense variant)
not provided
+2 more
GConflicting classifications of pathogenicity
NDUFV1
(S47T +1 more)
Single nucleotide variant
(missense variant)
not provided
+1 more
GUncertain significance
NDUFV1
(S56P +1 more)
Single nucleotide variant
(missense variant)
not specified
+5 more
GConflicting classifications of pathogenicity
NDUFV1
(V106M +1 more)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency, nuclear type 1
+1 more
GUncertain significance
NDUFV1
(S167A +1 more)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency, nuclear type 1
+2 more
GUncertain significance
NDUFV1
(R267K +1 more)
Single nucleotide variant
(missense variant)
Leigh syndrome
+4 more
GConflicting classifications of pathogenicity
NDUFV1
(E291K +1 more)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency, nuclear type 1
GUncertain significance
LOC126861242, NDUFV1
(V400M +1 more)
Single nucleotide variant
(missense variant)
Inborn genetic diseases
+1 more
GUncertain significance
LOC126861242, NDUFV1
(R396W +1 more)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency, nuclear type 1
+2 more
GUncertain significance
LOC126861242, NDUFV1
(T423M +1 more)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency
+4 more
GPathogenic/Likely pathogenic
LOC126861242, NDUFV1
(R443W +1 more)
Single nucleotide variant
(missense variant)
Inborn genetic diseases
+2 more
GUncertain significance
LOC126861242, NDUFV1
(R460W +1 more)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency
+5 more
GUncertain significance
NDUFS8
(R2C)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency, nuclear type 1
+4 more
GConflicting classifications of pathogenicity
NDUFS8
(P7T)
Single nucleotide variant
(missense variant)
Leigh syndrome
+2 more
GConflicting classifications of pathogenicity
TMEM126B
(Y107fs +3 more)
Microsatellite
(frameshift variant +1 more)
not provided
+2 more
GPathogenic/Likely pathogenic
TMEM126B
(M151V +4 more)
Single nucleotide variant
(missense variant +1 more)
not provided
+2 more
GUncertain significance
LOC130062145, NDUFV2
(A6G)
Single nucleotide variant
(missense variant)
not provided
+2 more
GConflicting classifications of pathogenicity
LOC130062145, NDUFV2
(R10L)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency, nuclear type 1
GUncertain significance
NDUFA11
(V69M)
Single nucleotide variant
(missense variant +1 more)
not provided
+1 more
GUncertain significance
LOC130065433, NDUFAF5
(N45K)
Single nucleotide variant
(missense variant +2 more)
Mitochondrial complex I deficiency, nuclear type 1
GUncertain significance
LOC130065433, NDUFAF5
(A59E)
Single nucleotide variant
(missense variant +2 more)
Mitochondrial complex I deficiency, nuclear type 1
GUncertain significance
NDUFAF5
(K109N)
Single nucleotide variant
(missense variant +2 more)
Inborn genetic diseases
+3 more
GPathogenic/Likely pathogenic
NDUFAF5
(S316fs +3 more)
Duplication
(frameshift variant +1 more)
not provided
+2 more
GConflicting classifications of pathogenicity
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