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Items: 75

Variation
Gene
(Protein Change)
Type
(Consequence)
ConditionClassification, Review status
NDUFS1
Single nucleotide variant
(3 prime UTR variant)
Mitochondrial complex I deficiency, nuclear type 1
+2 more
GUncertain significance
BCS1L, LOC129935609
Single nucleotide variant
(5 prime UTR variant +1 more)
Leigh syndrome
+3 more
GUncertain significance
BCS1L
(R56*)
Single nucleotide variant
(nonsense +3 more)
Mitochondrial complex III deficiency nuclear type 1
+5 more
GPathogenic
BCS1L
(R183H +2 more)
Single nucleotide variant
(missense variant +1 more)
not provided
+4 more
GPathogenic
NDUFA10
Deletion
(intron variant)
Mitochondrial complex I deficiency
+3 more
GBenign/Likely benign
NDUFA10
(E238K)
Single nucleotide variant
(missense variant +1 more)
not provided
+4 more
GBenign/Likely benign
SDHA
(R31*)
Single nucleotide variant
(nonsense)
Gastrointestinal stromal tumor
+9 more
GPathogenic/Likely pathogenic
SDHA
(S52F)
Single nucleotide variant
(missense variant)
Leigh syndrome
+7 more
GUncertain significance
SDHA
(A149T)
Single nucleotide variant
(missense variant +1 more)
Paragangliomas 5
+4 more
GUncertain significance
SDHA
(V150M)
Single nucleotide variant
(missense variant +1 more)
Paragangliomas 5
+6 more
GUncertain significance
SDHA
(Y205H +1 more)
Single nucleotide variant
(missense variant)
Paragangliomas 5
+5 more
GUncertain significance
SDHA
(R232C +1 more)
Single nucleotide variant
(missense variant)
Hereditary cancer-predisposing syndrome
+5 more
GUncertain significance
SDHA
(I235T +1 more)
Single nucleotide variant
(missense variant)
Dilated cardiomyopathy 1GG
+6 more
GUncertain significance
SDHA
(I247V +1 more)
Single nucleotide variant
(missense variant)
Dilated cardiomyopathy 1GG
+5 more
GConflicting classifications of pathogenicity
SDHA
(T271S +1 more)
Single nucleotide variant
(missense variant)
Paragangliomas 5
+5 more
GUncertain significance
SDHA
(Y301C +1 more)
Single nucleotide variant
(missense variant)
Hereditary cancer-predisposing syndrome
+5 more
GUncertain significance
SDHA
(I319L +1 more)
Single nucleotide variant
(missense variant)
not specified
+6 more
GConflicting classifications of pathogenicity
SDHA
(A331T +1 more)
Single nucleotide variant
(missense variant)
Leigh syndrome
+5 more
GConflicting classifications of pathogenicity
SDHA
(P332S +1 more)
Single nucleotide variant
(missense variant)
Mitochondrial complex II deficiency, nuclear type 1
+5 more
GUncertain significance
SDHA
(R352Q +1 more)
Single nucleotide variant
(missense variant)
Hereditary cancer-predisposing syndrome
+6 more
GConflicting classifications of pathogenicity
SDHA
(S384A +1 more)
Single nucleotide variant
(missense variant)
not provided
+5 more
GUncertain significance
SDHA
(A488T +1 more)
Single nucleotide variant
(missense variant)
Paragangliomas 5
+5 more
GUncertain significance
SDHA
Single nucleotide variant
(intron variant)
Leigh syndrome
+7 more
GBenign/Likely benign
SDHA
(R585W +1 more)
Single nucleotide variant
(missense variant +1 more)
Dilated cardiomyopathy 1GG
+6 more
GConflicting classifications of pathogenicity
SDHA
(E640G +2 more)
Single nucleotide variant
(missense variant)
Paragangliomas 5
+6 more
GConflicting classifications of pathogenicity
SDHA
(P658L +2 more)
Single nucleotide variant
(missense variant)
Dilated cardiomyopathy 1GG
+7 more
GConflicting classifications of pathogenicity
SDHA
Single nucleotide variant
(3 prime UTR variant)
Leigh syndrome
+5 more
GUncertain significance
NDUFS4
Single nucleotide variant
(intron variant)
Leigh syndrome
+2 more
GUncertain significance
NDUFS4
(K158fs)
Duplication
(3 prime UTR variant +2 more)
Mitochondrial complex I deficiency, nuclear type 1
+2 more
GPathogenic/Likely pathogenic
NDUFAF2
(W74*)
Single nucleotide variant
(nonsense)
not provided
+2 more
GPathogenic/Likely pathogenic
DLD
(R263H +3 more)
Single nucleotide variant
(missense variant)
not provided
+4 more
GConflicting classifications of pathogenicity
SURF1
(R192Q +1 more)
Single nucleotide variant
(missense variant)
Leigh syndrome
+2 more
GConflicting classifications of pathogenicity
SURF1
(R192W +1 more)
Single nucleotide variant
(missense variant)
Mitochondrial disease
+4 more
GPathogenic/Likely pathogenic
SURF1
Single nucleotide variant
(synonymous variant)
not provided
+4 more
GBenign/Likely benign
SURF1
(D170N +1 more)
Single nucleotide variant
(missense variant)
Charcot-Marie-Tooth disease type 4K
+3 more
GUncertain significance
SURF1
(R137W +1 more)
Single nucleotide variant
(missense variant)
Charcot-Marie-Tooth disease type 4K
+3 more
GUncertain significance
SURF1
(R123fs +1 more)
Deletion
(frameshift variant)
Charcot-Marie-Tooth disease type 4K
+3 more
GPathogenic
SURF1
(D108N)
Single nucleotide variant
(5 prime UTR variant +1 more)
Leigh syndrome
+4 more
GUncertain significance
SURF1
Indel
(5 prime UTR variant +1 more)
SURF1-related disorder
+4 more
GPathogenic
SURF1
Single nucleotide variant
(splice donor variant)
Leigh syndrome
+3 more
GPathogenic
SURF1
Deletion
(5 prime UTR variant +2 more)
Charcot-Marie-Tooth disease type 4K
+3 more
GPathogenic
COX15
(S44N)
Single nucleotide variant
(missense variant +2 more)
Cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency 2
+3 more
GUncertain significance
COX15
Single nucleotide variant
Cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency 2
+1 more
GUncertain significance
ECHS1
(G42fs)
Microsatellite
(frameshift variant)
See cases
+3 more
GPathogenic/Likely pathogenic
NDUFV1
(R59* +1 more)
Single nucleotide variant
(nonsense)
not provided
+2 more
GPathogenic
LOC126861242, NDUFV1
Single nucleotide variant
(intron variant)
Leigh syndrome
+2 more
GPathogenic/Likely pathogenic
LOC126861242, NDUFV1
(T423M +1 more)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency
+4 more
GPathogenic/Likely pathogenic
NDUFS8
(R2C)
Single nucleotide variant
(missense variant)
Mitochondrial complex I deficiency, nuclear type 1
+4 more
GConflicting classifications of pathogenicity
NDUFS8
(P79L)
Single nucleotide variant
(missense variant)
not provided
+1 more
GLikely pathogenic
FOXRED1
(Q232*)
Single nucleotide variant
(nonsense +1 more)
FOXRED1-related disorder
+3 more
GPathogenic
FOXRED1
(R252C)
Single nucleotide variant
(missense variant +1 more)
Leigh syndrome
+3 more
GConflicting classifications of pathogenicity
FOXRED1
(L391V)
Single nucleotide variant
(missense variant +1 more)
Mitochondrial complex I deficiency, nuclear type 1
+2 more
GConflicting classifications of pathogenicity
MTFMT
(S209L)
Single nucleotide variant
(missense variant)
Inborn genetic diseases
+10 more
GPathogenic
SCO1
(I263V)
Single nucleotide variant
(missense variant)
Leigh syndrome
+3 more
GUncertain significance
SCO1
(F102V)
Single nucleotide variant
(missense variant)
Leigh syndrome
+2 more
GUncertain significance
SCO1
Single nucleotide variant
(synonymous variant)
Mitochondrial complex IV deficiency, nuclear type 1
+4 more
GBenign/Likely benign
SCO1
(P87S)
Single nucleotide variant
(missense variant)
Mitochondrial complex 4 deficiency, nuclear type 4
+3 more
GUncertain significance
LOC112529895, SCO1
(R47Q)
Single nucleotide variant
(missense variant)
Leigh syndrome
+3 more
GUncertain significance
COX10
Single nucleotide variant
(5 prime UTR variant)
Leigh syndrome
+3 more
GUncertain significance
COX10
Single nucleotide variant
(5 prime UTR variant)
Leigh syndrome
+2 more
GUncertain significance
COX10
(R58H)
Single nucleotide variant
(missense variant)
Mitochondrial complex 4 deficiency, nuclear type 3
+4 more
GUncertain significance
COX10
(P104L)
Single nucleotide variant
(missense variant)
Leigh syndrome
+3 more
GConflicting classifications of pathogenicity
COX10
(D132Y)
Single nucleotide variant
(missense variant)
Leigh syndrome
+3 more
GUncertain significance
COX10, LOC105943586
(P246S)
Single nucleotide variant
(missense variant)
Mitochondrial complex IV deficiency, nuclear type 1
+2 more
GUncertain significance
COX10
(C343R)
Single nucleotide variant
(missense variant)
not provided
+3 more
GUncertain significance
COX10
(R354Q)
Single nucleotide variant
(missense variant)
not provided
+4 more
GUncertain significance
COX10
(A390V)
Single nucleotide variant
(missense variant)
Mitochondrial complex 4 deficiency, nuclear type 3
+4 more
GUncertain significance
COX10
(G396S)
Single nucleotide variant
(missense variant)
Leigh syndrome
+1 more
GUncertain significance
COX10
(R398C)
Single nucleotide variant
(missense variant)
Leigh syndrome
+3 more
GUncertain significance
NDUFAF5
Single nucleotide variant
(intron variant)
Mitochondrial complex 1 deficiency, nuclear type 16
+2 more
GUncertain significance
NDUFAF5
(T178M +3 more)
Single nucleotide variant
(missense variant +1 more)
Inborn genetic diseases
+1 more
GConflicting classifications of pathogenicity
NDUFAF5
(A137T +3 more)
Single nucleotide variant
(missense variant +1 more)
Mitochondrial complex 1 deficiency, nuclear type 16
GUncertain significance
MT-TL1
Single nucleotide variant
Cerebral palsy
+12 more
GPathogenic/Likely pathogenic
MT-ND4
Single nucleotide variant
Seizure
+2 more
GUncertain significance
MT-CYB
Single nucleotide variant
Leigh syndrome
+3 more
GUncertain significance
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