Leber Hereditary Optic Neuropathy malady

NIH Rare Diseases: Leber hereditary optic neuropathy (LHON) is an inherited form of vision loss. Although this condition usually begins in a person's teens or twenties, rare cases may appear in early childhood or later in adulthood. For unknown reasons, males are affected much more often than females. This condition is caused by mutations in the MT-ND1, MT-ND4, MT-ND4L, and MT-ND6 genes.³⁰

MalaCards: Leber Hereditary Optic Neuropathy, also known as LHON, is related to leber hereditary optic neuropathy with dystonia and melas syndrome. An important gene associated with Leber Hereditary Optic Neuropathy is MT-ND6 (mitochondrially encoded NADH dehydrogenase 6), and among its related pathways are Metformin Pathway, Pharmacodynamic and Pathway_PA165980337. The compounds 8-oxo-dg and myxothiazol have been mentioned in the context of this disorder. Affiliated tissues include whole blood, t cells and b cells, and related mouse phenotypes are muscle and vision/eye.

Genetics Home Reference: Leber hereditary optic neuropathy (LHON) is an inherited form of vision loss. Although this condition usually begins in a person's teens or twenties, rare cases may appear in early childhood or later in adulthood. For unknown reasons, males are affected much more often than females.¹⁷

GeneReviews summary for lhon

Aliases & Descriptions:

leber hereditary optic neuropathy 6 15 30 17 8 lhon 15 30 16 17 hereditary optic neuroretinopathy 15 16 17 leber optic atrophy 30 16 33 leber's hereditary optic neuropathy 6 16 leber's optic neuropathy 15 16 leber's optic atrophy 6 15 leber’s disease 30 16 lebers hereditary optic neuropathy 32

leber's optic atrophy (disorder) 6 optic atrophy, hereditary, leber 43 disorder of the optic nerve 43 leber congenital amaurosis 43 optic atrophy, leber type 30 optic atrophy, leber's 6 leber's disease 15 optic atrophy 43

Diseases related to leber hereditary optic neuropathy by text searches and GeneDecks gene sharing:

(show top 50)    (show all 377)

id	Related Disease	Score	Top Affiliating Genes
1	leber hereditary optic neuropathy with dystonia	35.6	MT-ND4, MT-ND6
2	melas syndrome	33.0	MT-ND5, MT-ND4, MT-CO3, MT-ND6, NDUFS4
3	open-angle glaucoma	32.4	MYOC, GSTM1, GSTT1, OPA1
4	behr syndrome	32.2	OPA1, OPA3
5	optic atrophy	31.8	SDHA, MT-ND5, MT-ND4, MT-ND1, MT-ND6, OPA1
6	optic neuritis	31.6	MT-ND5, MT-ND4, MT-CO3, MT-ND1, MT-ND2, MT-ND6
7	neuritis	31.6	MT-ND5, MT-ND4, MT-CO3, MT-ND1, MT-ND2, MT-ND6
8	alcoholism	31.3	TP53, ADH1B
9	optic nerve disease	31.3	MYOC, MT-ND4, MT-ND6, NDUFA1, OPA1
10	spasticity	30.9	SDHA, MT-ND4, MTHFR, MT-ATP6, MT-ND1, MT-ND6
11	cataract	29.1	MYOC, MTHFR, MT-CYB, GSR, GSTM1, GSTT1
12	sensorineural hearing loss	28.6	MT-ND5, MT-CO1, MTHFR, MT-ATP6, MT-ND6, GSTM1
13	hearing loss	28.4	MT-ND5, MT-CO1, MTHFR, MT-ATP6, MT-ND1, MT-ND6
14	neurodegeneration	28.4	GSR, CAT, AIFM1, TP53, NQO1, NOS3
15	blindness	28.2	MYOC, MT-ND5, MT-ND4, MTHFR, MT-ND1, MT-ND6
16	ataxia	27.1	MT-ND3, MT-ATP6, MT-ND1, XDH, GSR, FAS
17	peripheral neuropathy	27.0	MTHFR, MT-CO3, MT-CYB, MT-ND1, MT-ND2, GSTM1
18	glaucoma	27.0	MYOC, PARL, MT-ND4, MTHFR, MT-ATP6, MT-CO3
19	ptosis	26.4	FAS, AIFM1, TP53, NOS3, OPA1, SOD1
20	multiple sclerosis	25.7	MT-ND5, MT-ND4, MTHFR, MT-ATP6, MT-CO3, MT-CYB
21	retinitis	25.6	MYOC, MT-ND5, MT-ND4, MTHFR, MT-ATP6, MT-CYB
22	neuropathy	25.6	MYOC, PARL, SDHA, MT-TT, MT-ND5, MT-ND4L
23	diabetes mellitus	25.1	MT-ND5, MT-CO1, MT-ND4, MTHFR, MT-ND1, XDH
24	neuronitis	21.5	MYOC, MT-CO1, MT-CO3, MT-ND1, MT-ND2, XDH
25	mitochondrial encephalomyopathy lactic acidosis and stroke-like episodes	13.8	MT-ND5, MT-ND4, MT-ND1, MT-ND6
26	cortical blindness	13.8	MT-ND5, MT-ND4, MT-ND1, MT-ND6
27	leigh-like syndrome	13.8	MT-CO3, NDUFS4, OPA3
28	mitochondrial encephalomyopathy	13.7	MT-CO1, MT-ATP6, MT-CO3
29	kearns-sayre syndrome	13.7	SDHA, MT-ND5, MT-ATP6, MT-CYB, MT-ND6
30	lactic acidosis	13.5	MT-ND5, MT-ND4, MT-CO3, MT-ND1, NDUFS4, SOD2
31	echinococcosis	13.4	MT-CO1, MT-ATP6, XDH, IVNS1ABP
32	mitochondrial complex i deficiency	13.3	FOXRED1, NDUFAF5
33	brain cancer	13.3	GSTM1, GSTT1, NQO1
34	pthirus pubis infestation	13.3	MT-CO1, CAT, NPS
35	ariboflavinosis	13.3	MTHFR, GSR
36	mitochondrial dna-associated leigh syndrome and narp	13.1	MT-ND5, MT-ND3, MT-ND4, MT-ATP6, MT-CO3, MT-ND1
37	neuropathy ataxia retinitis pigmentosa syndrome	13.1	MT-ND5, MT-ND3, MT-ND4, MT-ATP6, MT-CO3, MT-ND1
38	hypertrophic cardiomyopathy	13.1	MT-CO1, MT-CO3, MT-CYB, MT-ND1, NOS3, NDUFS4
39	arterial occlusive disease	13.1	MTHFR, GSR, CYBA, CS
40	methemoglobinemia	13.1	MT-CYB, GSR, SOD1
41	sudden infant death syndrome	13.0	MT-ND3, MT-CYB, MT-ND1, GSTM1, GSTT1
42	chronic granulomatous disease	13.0	MT-CYB, CAT, CYBA, SOD1
43	asbestosis	13.0	GSTM1, GSTT1, CAT, SOD2
44	spermatic cord torsion	13.0	CAT, NOS3, SOD2
45	chronic obstructive pulmonary disease	12.9	XDH, GSR, GSTM1, GSTT1, EPHX1
46	hypotonia	12.9	MT-CO1, MT-CO3, AIFM1, NDUFS4, NDUFV1, SOD1
47	drug-induced hepatitis	12.9	GSTM1, GSTT1, CAT
48	diabetic cataract	12.9	GSR, CAT, SOD1
49	alcoholic liver cirrhosis	12.9	XDH, GSTM1, GSTT1, SOD2, ADH1B
50	digestive diseases	12.8	XDH, CAT, SOD1

Approved drugs:

Drug clinical trials:

Genetic tests related to leber hereditary optic neuropathy:

id	Genetic test	Affiliating Genes
1	Leber Hereditary Optic Neuropathy clinical/research	MT-ND5, MT-ND4L, MT-ND4, MT-ATP6, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND6

MalaCards organs/tissues related to leber hereditary optic neuropathy:

²²

MGI Mouse Phenotypes related to leber hereditary optic neuropathy:

²⁵

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	muscle phenotype	MP:0005369	7.9	SOD1, OPA3, OPA1, NDUFS4, NOS3, TP53
2	vision/eye phenotype	MP:0005391	7.2	MYOC, SOD2, SOD1, OPN4, OPA3, OPA1
3	cellular phenotype	MP:0005384	5.8	NQO1, NOS3, NDUFS4, OPA1, OPA3, OPN4
4	homeostasis/metabolism phenotype	MP:0005376	5.4	NOS3, NDUFS4, TFPI, OPA1, OPA3, OPN4

Articles related to leber hereditary optic neuropathy:

(show top 50)    (show all 92)

id	Title	Authors	Year	Affiliating Genes
1	Leber's hereditary optic neuropathy is associated wit h the T12338C mutation in mitochondrial ND5 gene in six Han Chinese families. (21131053)	Liu X.L.... Guan M.X.	2011	MT-ND5
2	Mitochondrial haplogroup background may influence Sou theast Asian G11778A Leber hereditary optic neuropathy. (21398275)	Kaewsutthi S.... Lertrit P.	2011	MT-ND4
3	The mitochondrial tRNA(Thr) A15951G mutation may be associated with Leber's hereditary optic neuropathy in two Chinese families]. (21983721)	Zhang Y.... Guan M.X.	2011	MT-TT
4	The mitochondrial ND5 T12338C mutation may be associ ated with Leber's hereditary optic neuropathy in two Chinese families]. (21482521)	Ji Y.C.... Guan M.X.	2011	MT-ND5
5	Variation in OPA1 does not explain the incomplete pen etrance of Leber hereditary optic neuropathy. (21203403)	Hudson G.... Chinnery P.F.	2010	OPA1
6	Evaluation of serum levels of SOD and MDA in patient s with Leber's hereditary optic neuropathy carrying the mitochondrial DNA G1177 8A mutation (20021885)	Liu Z.... Qu J.	2009	XDH
7	Leber's hereditary optic neuropathy is associated wit h mitochondrial ND6 T14502C mutation. (19732751)	Zhao F.... Guan M.X.	2009	MT-ND6
8	Comparison of retinal nerve fibre layers between 11778 and 14484 mutations in Leber's hereditary optic neuropathy. (19247386)	Seo J.H.... Park S.S.	2009	MT-ND4, MT-ND6
9	Leber hereditary optic neuropathy mutations in the ND6 subunit of mitochondrial complex I affect ubiquinone reduction kinetics in a bacterial model of the enzyme. (17894548)	Patsi J.... Hassinen I.E.	2008	NDUFS4, NDUFA5, MT-ND4
10	Phosphorylated neurofilament heavy chain is a marker of neurodegeneration in Leber hereditary optic neuropathy (LHON). (19104679)	Guy J.... Sadun A.A.	2008	NEFH
11	Mitochondrial DNA haplogroups M7b1'2 and M8a affect clinical expression of leber hereditary optic neuropathy in Chinese families with the m.11778G-->a mutation. (19026397)	Ji Y.... Yao Y.G.	2008	MT-ND5, MT-ATP6
12	The mitochondrial tRNA(Glu) A14693G mutation may influence the phenotypic manifestation of ND1 G3460A mutation in a Chinese family with Leber's hereditary optic neuropathy. (17434142)	Tong Y.... Guan M.X.	2007	MT-ND1
13	The NDUFB11 gene is not a modifier in Leber hereditary optic neuropathy. (17292333)	Petruzzella V.... Santorelli F.M.	2007	NDUFB11
14	The coexistence of mitochondrial ND6 T14484C and 12S rRNA A1555G mutations in a Chinese family with Leber's hereditary optic neuropathy and hearing loss. (17452034)	Wei Q.P.... Guan M.X.	2007	MT-ND6
15	Leber's hereditary optic neuropathy is associated with the mitochondrial ND4 G11696A mutation in five Chinese families. (16364244)	Zhou X.... Guan M.X.	2006	MT-ND4
16	Mitochondrial DNA mutations with Leber's hereditary optic neuropathy in Japanese patients with open-angle glaucoma. (16604388)	Inagaki Y.... Fukuchi T.	2006	MT-ND4, MT-ND1, MT-ND6
17	Antioxidant defences in cybrids harboring mtDNA mutations associated with Leber's hereditary optic neuropathy. (15720387)	Floreani M.... Dabbeni-Sala F.	2005	SOD1, SOD2, GSR
18	Genetic variants of TP53 and EPHX1 in Leber's hereditary optic neuropathy and their relationship to age at onset. (15838728)	Ishikawa K.... Mashima Y.	2005	TP53, NOS3, SOD2
19	Leber's hereditary optic neuropathy with molecular characterization in two Indian families. (16137960)	Verma I.C.... Grover A.K.	2005	MT-ND4, MT-ND1
20	Caspase-independent death of Leber's hereditary optic neuropathy cybrids is driven by energetic failure and mediated by AIF and Endonuclease G. (16151635)	Zanna C.... Rugolo M.	2005	AIFM1, ENDOG
21	Clinical features and the mutation of Leber's hereditary optic neuropathy in Chinese patients (15952130)	Wang Y.... Zhang Q.J.	2005	MT-ND4, MT-ND6, MT-ND1
22	Lack of association between Leber's hereditary optic neuropathy primary point mutations and multiple sclerosis in Iran. (14671420)	Houshmand M.... Lotfi J.	2004	MT-ND4, MT-ND1, MT-ND6
23	The ND1 gene of complex I is a mutational hot spot for Leber's hereditary optic neuropathy. (15505787)	Valentino M.L.... Carelli V.	2004	MT-ND1, MT-ND6
24	A mutational hot spot in the mitochondrial ND6 gene in patients with Leber's hereditary optic neuropathy. (11931086)	Luberichs J.... Fauser S.	2002	MT-ND6
25	Leber's hereditary optic neuropathy: clinical and molecular genetic results in a patient with a point mutation at np T11253C (isoleucine to threonine) in the ND4 gene and spontaneous recovery. (12271374)	Leo-Kottler B.... Fauser S.	2002	MT-ND4, MT-ND1, MT-ND6
26	Confirmation of the 14568 mutation in the mitochondrial ND6 gene as causative in Leber's hereditary optic neuropathy. (12324878)	Fauser S.... Luberichs J.	2002	MT-ND6
27	The mitochondrial ND6 gene is a hot spot for mutations that cause Leber's hereditary optic neuropathy. (11133798)	Chinnery P.F.... Howell N.	2001	MT-ND6
28	Novel mutation in RP2 gene in two brothers with X-linked retinitis pigmentosa and mtDNA mutation of leber hereditary optic neuropathy who showed marked differences in clinical severity. (11020419)	Mashima Y.... Shimizu N.	2000	RP2
29	Functional consequences of the 3460-bp mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy. (10426140)	Cock H.R.... Schapira A.H.	1999	MT-ND1
30	Biochemical features of mtDNA 14484 (ND6/M64V) point mutation associated with Leber's hereditary optic neuropathy. (10072046)	Carelli V.... Degli Esposti M.	1999	MT-CYB
31	Leber's hereditary optic neuropathy: clinical and molecular genetic findings in a patient with a new mutation in the ND6 gene. (10447650)	Besch D.... Wissinger B.	1999	MT-ND6
32	Secondary mutations of mitochondrial DNA in Japanese patients with Leber's hereditary optic neuropathy. (10520236)	Matsumoto M.... Kanai A.	1999	MT-ND1
33	Childhood Leber's hereditary optic neuropathy (ND1/3460) with visual recovery. (9831004)	Pezzi P.P.... Leuzzi V.	1998	NDUFA5
34	The nuoM arg368his mutation in NADH:ubiquinone oxidoreductase from Rhodobacter capsulatus: a model for the human nd4-11778 mtDNA mutation associated with Leber's hereditary optic neuropathy. (9685604)	Lunardi J.... Issartel J.P.	1998	NDUFS4, MT-ND4
35	Leber's hereditary optic neuropathy in Indonesia: two families with the mtDNA 11778G>A and 14484T>C mutations. (9452107)	Sudoyo H.... Marzuki S.	1998	MT-ND4, MT-ND6
36	High incidence of visual recovery among four Japanese patients with Leber's hereditary optic neuropathy with the 14484 mutation. (9176781)	Yamada K.... Oguchi Y.	1997	MT-ND6
37	Fast capillary electrophoresis-laser induced fluoresc ence analysis of ligase chain reaction products: human mitochondrial DNA point mutations causing Leber's hereditary optic neuropathy. (9034769)	Muth J.... Karger B.L.	1996	TFPI
38	Catalytic activity of complex I in cell lines that possess replacement mutations in the ND genes in Leber's hereditary optic neuropathy. (8706709)	Majander A.... Wikstrom M.	1996	CS, MT-ND4, MT-ND5
39	Genetic and biochemical impairment of mitochondrial complex I activity in a family with Leber hereditary optic neuropathy and hereditary spastic dystonia. (8644732)	de Vries D.D.... van Oost B.A.	1996	NDUFS4, MT-ND4, MT-ND6
40	Phylogenetic analysis of Leber's hereditary optic neuropathy mitochondrial DNA's indicates multiple independent occurrences of the common mutations. (8680405)	Brown M.D.... Wallace D.C.	1995	MT-ND4L
41	Leber's hereditary optic neuropathy (LHON)-related mitochondrial DNA sequence changes in italian patients presenting with sporadic bilateral optic neuritis. (8593537)	Sartore M.... Arbustini E.	1995	MT-ND4
42	Molecular genetic analysis of Leber's hereditary optic neuropathy with the 3460 mutation in Japanese pedigrees (7611010)	Hiida Y.... Oguchi Y.	1995	MT-ND1
43	A mitochondrial DNA mutation at nucleotide pair 14459 of the NADH dehydrogenase subunit 6 gene associated with maternally inherited Leber hereditary optic neuropathy and dystonia. (8016139)	Jun A.S.... Wallace D.C.	1994	MT-ND6
44	When does bilateral optic atrophy become Leber hereditary optic neuropathy? (8213825)	Howell N.... Poulton J.	1993	MT-ND5
45	Intrafamilial variation in Leber hereditary optic neuropathy revealed by direct mutation analysis. (8448903)	Cavelier L.... Dahl N.	1993	MT-ND4
46	A mitochondrial DNA variant, identified in Leber hereditary optic neuropathy patients, which extends the amino acid sequence of cytochrome c oxidase subunit I. (1322638)	Brown M.D.... Wallace D.C.	1992	MT-CO1
47	High frequency of mitochondrial ND4 gene mutation in Japanese pedigrees with Leber hereditary optic neuropathy. (1635296)	Nakamura M.... Yamamoto M.	1992	MT-ND4
48	Cytochrome b mutations in Leber hereditary optic neuropathy. (1764087)	Johns D.R.... Neufeld M.J.	1991	MT-CYB
49	Detection of the G to A mitochondrial DNA mutation at position 11778 in German families with Leber's hereditary optic neuropathy. (1959931)	Kormann B.A.... Leo-Kottler B.	1991	MT-ND4
50	An example of Leber hereditary optic neuropathy not involving a mutation in the mitochondrial ND4 gene. (2121024)	Howell N.... McCullough D.	1990	MT-ND4

Genes related to leber hereditary optic neuropathy according to GeneCards:

(show top 50)    (show all 63)

id	Symbol	Description	Score	GeneCards Section Context
1	MT-ND6	mitochondrially encoded NADH dehydrogenase 6	11.1	Publications
2	MT-ND4	mitochondrially encoded NADH dehydrogenase 4	11.1	Publications
3	NDUFA1	NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa	11.0	Publications
4	MT-CYB	mitochondrially encoded cytochrome b	11.0	Publications
5	NDUFS4	NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase)	11.0	Publications
6	MT-ND1	mitochondrially encoded NADH dehydrogenase 1	11.0	Publications
7	EPHX1	epoxide hydrolase 1, microsomal (xenobiotic)	11.0	Publications
8	OPA1	optic atrophy 1 (autosomal dominant)	11.0	Publications
9	MYOC	myocilin, trabecular meshwork inducible glucocorticoid response	11.0
10	MT-ND5	mitochondrially encoded NADH dehydrogenase 5	11.0	Publications
11	MT-CO3	mitochondrially encoded cytochrome c oxidase III	11.0	Publications
12	MT-CO1	mitochondrially encoded cytochrome c oxidase I	11.0	Publications
13	MT-TT	mitochondrially encoded tRNA threonine	11.0	Publications
14	RHBDL1	rhomboid, veinlet-like 1 (Drosophila)	11.0
15	NDUFA5	NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 5, 13kDa	11.0	Publications
16	MT-ATP6	mitochondrially encoded ATP synthase 6	11.0	Publications
17	NDUFB11	NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11, 17.3kDa	11.0	Publications
18	NDUFAF4	NADH dehydrogenase (ubiquinone) complex I, assembly factor 4	11.0
19	NDUFA11	NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 11, 14.7kDa	11.0
20	FOXRED1	FAD-dependent oxidoreductase domain containing 1	11.0
21	NDUFAF3	NADH dehydrogenase (ubiquinone) complex I, assembly factor 3	11.0
22	NDUFS7	NADH dehydrogenase (ubiquinone) Fe-S protein 7, 20kDa (NADH-coenzyme Q reductase)	11.0
23	NPS	neuropeptide S	11.0
24	ENDOG	endonuclease G	11.0	Publications
25	MRPL47	mitochondrial ribosomal protein L47	11.0
26	NDUFS1	NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase)	11.0	Publications
27	TRAPPC2	trafficking protein particle complex 2	11.0	Publications
28	NEFH	neurofilament, heavy polypeptide	11.0	Publications
29	RP2	retinitis pigmentosa 2 (X-linked recessive)	11.0	Publications
30	CS	citrate synthase	11.0	Publications
31	NDUFV1	NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa	11.0
32	PARL	presenilin associated, rhomboid-like	11.0
33	OPN4	opsin 4	11.0
34	NDUFS2	NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase)	11.0
35	ADH1B	alcohol dehydrogenase 1B (class I), beta polypeptide	11.0	Publications
36	TFPI	tissue factor pathway inhibitor (lipoprotein-associated coagulation inhibitor)	11.0	Publications
37	TMEM48	transmembrane protein 48	11.0	Disorders
38	SDHA	succinate dehydrogenase complex, subunit A, flavoprotein (Fp)	11.0	Publications
39	OPA3	optic atrophy 3 (autosomal recessive, with chorea and spastic paraplegia)	11.0
40	CYBA	cytochrome b-245, alpha polypeptide	11.0	Publications
41	IVNS1ABP	influenza virus NS1A binding protein	11.0
42	AIFM1	apoptosis-inducing factor, mitochondrion-associated, 1	11.0	Publications
43	SLC7A14	solute carrier family 7 (orphan transporter), member 14	11.0
44	NDUFAF2	NADH dehydrogenase (ubiquinone) complex I, assembly factor 2	11.0
45	PRKX	protein kinase, X-linked	11.0
46	GSR	glutathione reductase	11.0	Publications
47	NQO1	NAD(P)H dehydrogenase, quinone 1	11.0	Publications
48	NUBPL	nucleotide binding protein-like	11.0
49	GSTT1	glutathione S-transferase theta 1	11.0	Publications
50	XDH	xanthine dehydrogenase	11.0	Publications

Pathways related to leber hereditary optic neuropathy according to GeneDecks:

(show all 16)

id	Pathway	Score	Top Affiliating Genes
1	Metformin Pathway, Pharmacodynamic34	10.1	NDUFV1, NDUFS4, NDUFS2, NDUFS1, NDUFA11, NDUFA1
2	Pathway_PA16598033734	9.7	SOD1, CAT, GSR
3	Oxidative Stress Pathway (Erythrocyte)34	9.6	SOD1, CAT, GSR
4	Platinum Pathway, Pharmacokinetics/Pharmacodynamics34	9.6	SOD1, NQO1, GSTT1, GSTM1
5	Oxidative Stress Pathway (Erythrocyte)34	9.6	SOD1, CAT, GSR
6	Apoptosis Signaling Pathways37	9.1	FAS, ENDOG, AIFM1, TP53
7	Alzheimers disease20	9.1	MT-CO1, MT-ATP6, MT-CO3, MT-CYB, FAS, NDUFA1
8	Doxorubicin Pathway, Pharmacokinetics34	8.9	SOD1, NDUFS7, NDUFS2, NOS3, NQO1, CAT
9	Apoptosis20	8.8	PRKX, TP53, AIFM1, ENDOG, FAS
10	Amyotrophic lateral sclerosis (ALS)20	8.7	NEFH, SOD1, TP53, CAT
11	Doxorubicin Pathway (Cancer Cell), Pharmacodynamics34	8.5	SOD1, NOS3, NQO1, TP53, CAT, XDH
12	Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins.38	8.4	MT-ND1, MT-ND2, MT-ND6, NDUFA1, NDUFA11, NDUFA5
13	Huntingtons disease20	8.4	SDHA, MT-CO1, MT-ATP6, SOD2, MT-CO3, MT-CYB
14	Oxidative phosphorylation20	8.3	NDUFA11, NDUFA5, NDUFB11, NDUFS1, NDUFS2, NDUFS4
15	Parkinsons disease20	8.2	SDHA, MT-ND5, MT-ND4L, MT-ND3, MT-CO1, NDUFV1
16	Metabolic pathways20	6.7	SDHA, NDUFA1, NDUFA11, NDUFA5, NDUFS1, NDUFS2

Compounds related to leber hereditary optic neuropathy according to GeneDecks:

(show top 50)    (show all 160)

id	Compound	Score	Top Affiliating Genes
1	8-oxo-dg32	10.3	TP53
2	myxothiazol32 9 9	12.1	MT-CYB, NDUFS4, SOD2, XDH
3	succinate32	10.1	SDHA, MT-CO1, NDUFS4, SOD2, CS, MT-ND4
4	1,3-butadiene32	10.0	GSTM1, GSTT1, EPHX1, TFPI
5	apocynin32	10.0	NDUFS4, XDH, CAT, CYBA
6	h2o232	9.9	XDH, MT-CYB, AIFM1, CYBA, NQO1, NDUFS4
7	citrinin32	9.8	NDUFS4, SOD1, MT-CYB, XDH, CAT
8	semiquinone32	9.8	MT-CYB, NDUFA5, SOD1, CAT, NQO1
9	n acetylcysteine32	9.8	NQO1, CYBA, ENDOG, TP53
10	antimycin a32	9.7	NDUFS4, SOD1, CAT, MT-CYB, SOD2
11	propyl gallate32	9.6	GSR, CAT, SOD1, SOD2
12	pyrogallol32	9.5	XDH, CAT, SOD1, SOD2
13	citrate32	9.5	SOD2, NOS3, CAT, MT-CO3, NDUFS4, MT-CO1
14	quinone32 18	10.5	NDUFA5, GSTT1, GSTM1, MT-CYB, CAT, NQO1
15	gsts32	9.5	GSTM1, GSTT1, GSR, MTHFR, NQO1, EPHX1
16	oxypurinol32	9.5	XDH, CAT, NOS3, SOD1
17	3-amino-1,2,4-triazole32	9.4	SOD1, GSR, CAT, SOD2, XDH
18	fenton32	9.4	XDH, SOD2, SOD1, MT-CYB, GSR, CAT
19	gamma-glutamylcysteine32 9 18 9	12.4	GSR, EPHX1, SOD2, NQO1, CAT, GSTM1
20	glutathion32	9.4	EPHX1, SOD2, GSTM1, CAT, GSR, SOD1
21	hydroquinone32 18	10.4	SOD2, SOD1, CAT, MT-CYB, NQO1
22	dmpo32	9.4	XDH, CAT, SOD1, SOD2
23	alanine32	9.4	MYOC, TFPI, NQO1, MT-ND6, MT-ND2, MT-ND1
24	gssg32	9.4	SOD1, GSR, NDUFS4, GSTM1, CAT, SOD2
25	ubiquinone32	9.3	MT-ND4, MT-CYB, GSR, CAT, NQO1, CS
26	6-hydroxydopamine32	9.3	SOD2, SOD1, NDUFS4, CAT, NQO1, GSR
27	valine32	9.2	MT-ND6, SOD2, MT-ND5, MT-ND1, MT-ND4, MTHFR
28	ascorbic acid32 18	10.1	SOD2, MYOC, FAS, AIFM1, GSTT1, NQO1
29	allopurinol32 9 9	11.1	CAT, SOD2, SOD1, NOS3, NQO1, XDH
30	diphenyleneiodonium32	9.1	CAT, NDUFS4, XDH, CYBA, NOS3, SOD1
31	rotenone32	9.0	SOD1, NDUFA5, AIFM1, CAT, GSR, XDH
32	malondialdehyde32	8.9	CAT, GSTT1, GSTM1, SOD1, GSR, XDH
33	manganese superoxide32	8.9	CS, SOD2, SOD1, NQO1, CAT, GSTT1
34	menadione32 9 18 9	11.9	SOD2, NQO1, SOD1, MTHFR, GSR, XDH
35	bcnu32	8.8	NQO1, TP53, SOD2, GSTT1, GSR
36	dicoumarol32	8.8	SOD1, XDH, CAT, TP53, NQO1, SOD2
37	peroxynitrite32	8.8	GSR, SOD1, NOS3, NDUFS4, SOD2, CS
38	betacarotene32	8.7	MTHFR, GSR, GSTM1, CAT, SOD1, SOD2
39	4-hydroxynonenal32 18	9.6	CS, SOD2, SOD1, CAT, FAS, GSTM1
40	mitomycin c32	8.6	SOD2, TP53, NQO1, FAS, GSR, XDH
41	alpha tocopherol32	8.6	MTHFR, GSR, GSTM1, CAT, SOD2, SOD1
42	xanthine32 18	9.4	NQO1, CAT, XDH, NOS3, SOD1, SOD2
43	lactate32	8.1	MT-ND1, XDH, FAS, GSTM1, CAT, GSR
44	superoxide32 18	9.1	XDH, CYBA, NQO1, NOS3, NDUFS4, MT-CYB
45	oxygen32 18	8.9	SDHA, ENDOG, FAS, GSTT1, GSTM1, GSR
46	arsenite32 18	8.7	GSR, FAS, CAT, AIFM1, NQO1, SOD1
47	nadph32 18	8.7	MT-CYB, XDH, GSR, GSTT1, NOS3, CAT
48	iron32 18	8.6	SDHA, MT-ND4L, MT-CO1, MT-ND4, MT-ND1, SOD2
49	doxorubicin32 34 9 9	10.6	NQO1, CAT, FAS, GSTT1, GSTM1, GSR
50	nadh32 9 18 9	8.6	MT-CYB, MT-CO3, MT-ATP6, MTHFR, MT-ND4, MT-CO1

Cellular components related to leber hereditary optic neuropathy according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	mitochondrial intermembrane space	GO:005758	9.7	CAT, AIFM1, NDUFS1, OPA1
2	mitochondrial respiratory chain complex I	GO:005747	9.6	NDUFV1, NDUFS7, NDUFS4, NDUFS2, NDUFS1, NDUFB11
3	mitochondrial inner membrane	GO:005743	8.3	PARL, OPA1, NDUFV1, NDUFS7, NDUFS4, NDUFS2
4	mitochondrion	GO:005739	7.1	NDUFAF4, NDUFS2, NDUFS4, OPA3, NUBPL, SOD1

Biological processes related to leber hereditary optic neuropathy according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	apoptotic DNA fragmentation	GO:006309	9.9	ENDOG, AIFM1, SOD1
2	mitochondrial electron transport, NADH to ubiquinone	GO:006120	9.9	NDUFV1, NDUFS7, NDUFS4, NDUFS2, NDUFS1, NDUFA5
3	respiratory electron transport chain	GO:022904	9.6	NDUFV1, NDUFS7, NDUFS4, NDUFS2, NDUFS1, NDUFB11
4	superoxide metabolic process	GO:006801	9.4	SOD2, SOD1, CYBA
5	mitochondrial respiratory chain complex I assembly	GO:032981	8.5	AIFM1, NUBPL, NDUFS7, NDUFS4, NDUFAF6, NDUFAF5
6	small molecule metabolic process	GO:044281	7.2	NDUFB11, NDUFS1, NDUFS2, NDUFS4, NDUFS7, NDUFV1

Molecular functions related to leber hereditary optic neuropathy according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	superoxide dismutase activity	GO:004784	9.9	SOD1, SOD2
2	4 iron, 4 sulfur cluster binding	GO:051539	9.8	NUBPL, NDUFV1, NDUFS7, NDUFS2, NDUFS1
3	NADH dehydrogenase (ubiquinone) activity	GO:008137	9.7	NDUFA1, NDUFV1, NDUFS7, NDUFS4, NDUFS2, NDUFS1
4	electron carrier activity	GO:009055	9.2	NDUFS2, NDUFS1, NDUFAF2, CYBA, AIFM1, GSR
5	flavin adenine dinucleotide binding	GO:050660	9.0	NOS3, AIFM1, GSR, XDH, SDHA