Mitochondrial Encephalomyopathy malady

NINDS: Mitochondrial myopathies are a group of neuromuscular diseases caused by damage to the mitochondria?small, energy-producing structures that serve as the cells' "power plants." Nerve cells in the brain and muscles require a great deal of energy, and thus appear to be particularly damaged when mitochondrial dysfunction occurs. Some of the more common mitochondrial myopathies include Kearns-Sayre syndrome, myoclonus epilepsy with ragged-red fibers, and mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes. The symptoms of mitochondrial myopathies include muscle weakness or exercise intolerance, heart failure or rhythm disturbances, dementia, movement disorders, stroke-like episodes, deafness, blindness, droopy eyelids, limited mobility of the eyes, vomiting, and seizures. The prognosis for these disorders ranges in severity from progressive weakness to death. Most mitochondrial myopathies occur before the age of 20, and often begin with exercise intolerance or muscle weakness. During physical activity, muscles may become easily fatigued or weak. Muscle cramping is rare, but may occur. Nausea, headache, and breathlessness are also associated with these disorders.³¹

MalaCards: Mitochondrial Encephalomyopathy, also known as mitochondrial myopathies, is related to mitochondrial encephalomyopathy lactic acidosis and stroke-like episodes and melas syndrome. An important gene associated with Mitochondrial Encephalomyopathy is MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes), and among its related pathways are Citric acid cycle (TCA cycle) and Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins.. The drugs naloxone and nalmefene and the compounds myxothiazol and n acetylcysteine have been mentioned in the context of this disorder. Affiliated tissues include brain, heart and skeletal muscle, and related mouse phenotypes are muscle and cardiovascular system.

Wikipedia: Mitochondrial disease is a group of disorders caused by dysfunctional mitochondria, the organelles that...⁴⁴ more...

GeneReviews summary for mt-overview

Aliases & Descriptions:

mitochondrial encephalomyopathy 6 8 mitochondrial myopathies 15 16 17 31 32 43 mitochondrial encephalomyopathies 15 16 32 43 mitochondrial diseases 17 32 43 oxidative phosphorylation disorders 15 16 respiratory chain disorders 15 16

mitochondrial disorders 15 16 mitochondrial respiratory chain deficiencies 43 respiratory chain deficiency 32 mitochondrial myopathy 8 respiration disorders 43 mitochondrial disease 8

Diseases related to mitochondrial encephalomyopathy by text searches and GeneDecks gene sharing:

(show top 50)    (show all 234)

id	Related Disease	Score	Top Affiliating Genes
1	mitochondrial encephalomyopathy lactic acidosis and stroke-like episodes	34.6	MT-ND5, MT-TK, MT-TL1, MT-ND4, MT-ND1, MT-ND6
2	melas syndrome	33.7	MT-ND5, MT-TL1
3	lactic acidosis	31.5	MT-ND5, MT-ND4, MT-ND1, GHRH, SURF1, COX5A
4	mitochondrial complex i deficiency	31.2	MT-ND5, MT-ND1, MT-ND6, FH, NDUFA1, NDUFA8
5	sideroblastic anemia	31.1	MT-CO1, SUCLA2, COX5A, SOD2, CP
6	chronic progressive external ophthalmoplegia	30.1	SUCLA2, COX5A, POLG
7	kearns-sayre syndrome	28.9	MT-ND5, MT-CO2, MT-ATP6, MT-CYB, MT-ND6, COX5A
8	merrf syndrome	28.2	MT-ND5, COX5A, NDUFS4
9	friedreich ataxia	28.1	NDUFS4, SOD1, SOD2, POLG
10	respiratory failure	28.1	MB, NDUFS4, TK2, DNAH8, RYR1
11	cytochrome-c oxidase deficiency disease	27.9	FASTKD2, SURF1, COX5A
12	pearson syndrome	27.5	TRMU, POLG, NPTX2
13	optic atrophy	27.0	RARS2, MT-ND5, MT-ND4, MT-ND1, MT-ND6, CYCS
14	anemia	26.5	BCS1L, MT-CO1, MT-ATP6, MB, CAT, GH1
15	cataract	25.9	MT-CYB, FH, HSPD1, CAT, CYCS, RPS27A
16	peripheral neuropathy	25.9	MT-CYB, MT-ND1, CAT, SURF1, COX5A, POLG
17	encephalomyopathy	25.6	BLVRB, BCS1L, MT-TE, MT-ND5, MT-TR, MT-TK
18	seizures	25.5	MT-ND5, MT-CO1, MT-ND4, MT-ATP6, MT-ND1, MT-ND6
19	parkinson's disease	25.4	BLVRB, MT-ND5, MT-CYB, MT-ND1, CAT, GHRH
20	retinitis	23.6	BLVRB, MT-ND5, MT-ND4, MT-ATP6, MT-CYB, MT-ND1
21	ataxia	23.5	BCS1L, MT-TK, MT-TL1, MT-CO2, MT-ATP6, MT-ND1
22	diabetes mellitus	21.4	MT-TE, MT-ND5, MT-TL1, MT-CO1, MT-CO2, MT-ND4
23	neuropathy	19.4	BCS1L, MT-ND5, MT-CO1, MT-ND4, MT-ATP6, MT-CYB
24	myopathy	19.2	MT-ND5, MT-TK, MT-TL1, MT-CO1, MT-CO2, MT-ATP6
25	merrf/melas overlap syndrome	13.8	MT-TK, MT-TL1
26	optic nerve disease	13.7	MT-ND4, MT-ND6, NDUFA1
27	leber hereditary optic neuropathy with dystonia	13.7	MT-ND4, MT-ND6
28	combined oxidative phosphorylation deficiency	13.6	MRPS16, AIFM1, TSFM
29	myoclonus epilepsy	13.6	MT-ND5, MT-TK, MT-CO2, TRMU, COX5A
30	mitochondrial dna-associated leigh syndrome and narp	13.5	MT-ND5, MT-TK, MT-TL1, MT-ND4, MT-ATP6, MT-ND1
31	cortical blindness	13.4	MT-ND5, MT-ND4, MT-ND1, MT-ND6, POLG
32	coenzyme q10 deficiency disease	13.4	COQ2, TK2, POLG
33	isolated growth hormone deficiency	13.3	GH1, GHRH, POMC
34	rhabdomyolysis, cerivastatin-induced	13.3	MB, COX5A, RYR1
35	pili torti	13.3	BCS1L, CP
36	adrenal gland hyperfunction	13.3	GH1, GHRH, POMC
37	alpers syndrome	13.2	SUCLA2, TK2, POLG
38	lipodystrophy	13.1	MT-ND6, GH1, GHRH, CS, POLG
39	orthostatic hypotension	13.1	MT-ND5, MT-CYB, POMC
40	congenital heart defect	13.1	MB, CYCS, SOD2, CS, RYR1
41	spinal muscular atrophy	13.1	COX5A, NDUFS4, TK2, SOD1
42	zollinger-ellison syndrome	13.1	GHRH, DNAH8, POMC
43	aceruloplasminemia	13.1	SOD1, SOD2, CP
44	neuropathy ataxia retinitis pigmentosa syndrome	13.1	MT-ND5, MT-TK, MT-TL1, MT-ND4, MT-ATP6, MT-ND1
45	leukodystrophy	13.1	MT-ND5, MT-ND6, HSPD1, SURF1, NDUFA1, NDUFAF2
46	familial male-limited precocious puberty	13.0	BLVRB, GH1, GHRH, POMC
47	galactosemia	13.0	BCS1L, CS, POLG, CP, CPOX
48	neuroleptic malignant syndrome	13.0	MB, POMC, RYR1
49	ophthalmoplegia	13.0	SUCLA2, COX5A, TFAM, SOD2, POLG, RYR1
50	methemoglobinemia	13.0	BLVRB, MT-CYB, SOD1

Approved drugs:

Drug clinical trials:

Inferred drug relations via UMLS/NDF-RT:

^43 28 nalmefene, nalmefene hydrochloride, naloxone, naloxone hydrochloride

Genetic tests related to mitochondrial encephalomyopathy:

id	Genetic test	Affiliating Genes
1	Mitochondrial Encephalomyopathy clinical/research	MT-TL2, MT-TT, MT-TV, MT-ND5, MT-TC, MT-TM, MT-TR, MT-TF, MT-TK, MT-TS1 (show all 22) MT-TD, MT-TL1, MT-ND4, MT-TQ, MT-TS2, MT-ATP6, MT-ND1, MT-TH, MT-TW, MT-TY, MT-ND6, MT-TA

MalaCards organs/tissues related to mitochondrial encephalomyopathy:

²²

MGI Mouse Phenotypes related to mitochondrial encephalomyopathy:

²⁵

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	muscle phenotype	MP:0005369	7.9	POLG, SOD2, SOD1, TFAM, TK2, NDUFS4
2	cardiovascular system phenotype	MP:0005385	6.7	MT-CO1, CP, RYR1, POMC, POLG, SOD2

Articles related to mitochondrial encephalomyopathy:

(show all 37)

id	Title	Authors	Year	Affiliating Genes
1	Human mitochondrial leucyl-tRNA synthetase corrects m itochondrial dysfunctions due to the tRNALeu(UUR) A3243G mutation, associated w ith mitochondrial encephalomyopathy, lactic acidosis, and stroke-like symptoms and diabetes. (20194621)	Li R.... Guan M.X.	2010	LARS2
2	Severe X-linked mitochondrial encephalomyopathy assoc iated with a mutation in apoptosis-inducing factor. (20362274)	Ghezzi D.... Zeviani M.	2010	AIFM1
3	Infantile mitochondrial encephalomyopathy with unusual phenotype caused by a novel BCS1L mutation in an isolated complex III-deficient patient. (19162478)	BlA!zquez A.... Ugalde C.	2009	BCS1L
4	FASTKD2 nonsense mutation in an infantile mitochondrial encephalomyopathy associated with cytochrome c oxidase deficiency. (18771761)	Ghezzi D.... Zeviani M.	2008	COX5A, FASTKD2
5	Mitochondrial encephalomyopathy with elevated methylmalonic acid is caused by SUCLA2 mutations. (17287286)	Ostergaard E.... Schwartz M.	2007	SUCLA2
6	X-linked NDUFA1 gene mutations associated with mitochondrial encephalomyopathy. (17262856)	Fernandez-Moreira D.... Arenas J.	2007	NDUFS4, NDUFA1
7	Detection of common disease-causing mutations in mitochondrial DNA (mitochondrial encephalomyopathy, lactic acidosis with stroke-like episodes MTTL1 3243 A>G and myoclonic epilepsy associated with ragged-red fibers MTTK 8344A>G) by real-time polymerase chain reaction. (16645216)	Fan H.... Farber R.A.	2006	MT-TL1, MT-TK
8	Myostatin expression in muscular dystrophies and mitochondrial encephalomyopathies. (16996410)	Markert C.... Childers M.K.	2006	MSTN
9	Myostatin expression in muscular dystrophies and mitochondrial encephalomyopathies. (16638502)	Castro-Gago M.... Devesa J.	2006	MSTN
10	Pathology of mitochondrial encephalomyopathies. (16018150)	Sarnat H.B.... MarA-n-GarcA-a J.	2005	CS
11	Molecular epidemiology of childhood mitochondrial encephalomyopathies in a Finnish population: sequence analysis of entire mtDNA of 17 children reveals heteroplasmic mutations in tRNAArg, tRNAGlu, and tRNALeu(UUR) genes. (15286228)	Uusimaa J.... Majamaa K.	2004	MT-TR, MT-TE
12	Different tissue distribution of a mitochondrial DNA duplication and the corresponding deletion in a patient with a mild mitochondrial encephalomyopathy: deletion in muscle, duplication in blood. (15036329)	Houshmand M.... Holme E.	2004	MT-CO1
13	Overexpressions of myoglobin and antioxidant enzymes in ragged-red fibers of skeletal muscle from patients with mitochondrial encephalomyopathy. (14506721)	Kunishige M.... Matsumoto T.	2003	SOD1, SOD2, CAT
14	Apoptosis and ROS detoxification enzymes correlate with cytochrome c oxidase deficiency in mitochondrial encephalomyopathies. (11312605)	Di Giovanni S.... Servidei S.	2001	COX5A
15	The incidence of mitochondrial encephalomyopathies in childhood: clinical features and morphological, biochemical, and DNA anbormalities. (11261513)	Darin N.... Tulinius M.	2001	SURF1
16	Mitochondrial encephalomyopathy and complex III deficiency associated with a stop-codon mutation in the cytochrome b gene. (11047755)	Keightley J.A.... Kennaway N.G.	2000	MT-CYB
17	Abnormal levels of human mitochondrial transcription factor A in skeletal muscle in mitochondrial encephalomyopathies. (11382203)	Siciliano G.... Iudice A.	2000	COX5A, TFAM
18	Early mitochondrial encephalomyopathy due to complex IV deficiency consistent with Alpers-Huttenlocher syndrome: report of two cases (10637838)	Castro-Gago M.... Eiris-Punal J.	1999	CPOX
19	Muscle carnitine acetyltransferase and carnitine defi ciency in a case of mitochondrial encephalomyopathy. (10518284)	Melegh B.... MAches K.	1999	CRAT
20	An mtDNA mutation in the initiation codon of the cytochrome C oxidase subunit II gene results in lower levels of the protein and a mitochondrial encephalomyopathy. (10205264)	Clark K.M.... Turnbull D.M.	1999	MT-CO2
21	Treatment of mitochondrial encephalomyopathy with a combination of cytochrome C and vitamins B1 and B2. (9187476)	Tanaka J.... Okada S.	1997	CYCS
22	Histological determination of nitric oxide synthase (NOS) and NADPH-diaphorase in ragged-red fibers from patients with mitochondrial encephalomyopathies. (9171323)	Ohkoshi N.... Shoji S.	1997	BLVRB
23	Cytochrome c oxidase deficiency in fibroblasts of a patient with mitochondrial encephalomyopathy. (8918062)	Lee W.T.... Shen Y.Z.	1996	RYR1
24	Oxidative damage to skeletal muscle DNA from patients with mitochondrial encephalomyopathies. (8836981)	Mitsui T.... Saito S.	1996	SOD1
25	Focal cytochrome c oxidase deficiency in the brain and dorsal root ganglia in a case with mitochondrial encephalomyopathy (tRNA(Ile) 4269 mutation): histochemical, immunohistochemical, and ultrastructural study. (7595643)	Kaido M.... Yanagihara T.	1995	COX5A
26	Superoxide dismutases of muscle in mitochondrial encephalomyopathies. (7565923)	Ohkoshi N.... Yoshizawa K.	1995	SOD2
27	Gonadal dysfunction in mitochondrial encephalomyopathies. (8542917)	Chen C.M.... Huang C.C.	1995	POMC
28	Free radical, lipid peroxide and antioxidant in mitochondrial encephalomyopathy (7955720)	Ihara Y.... Mori A.	1994	SOD1, CP
29	Decreased synthesis and inefficient mitochondrial import of hsp60 in a patient with a mitochondrial encephalomyopathy. (7986829)	Huckriede A.... Agsteribbe E.	1994	HSPD1
30	Quantitative evaluation of electron transport system proteins in mitochondrial encephalomyopathy. (8386896)	Haginoya K.... TADA K.	1993	COX5A
31	Immunolocalization of heat shock proteins in ragged-r ed fibers of patients with mitochondrial encephalomyopathies. (8101114)	Sparaco M.... Bonilla E.	1993	RPS27A
32	A molecular genetic study of focal histochemical defects in mitochondrial encephalomyopathies. (1606473)	Hammans S.R.... Harding A.E.	1992	COX5A
33	An autopsy case of mitochondrial encephalomyopathy with prominent degeneration in olivo-ponto-cerebellar system. (1792871)	Kageyama Y.... Miyoshi K.	1991	NDUFS4
34	Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) associated with hypothalamo-pituitary hypofunctio n--a case report (2060243)	Ishii A.... Kanazawa I.	1991	GH1, GHRH
35	Mitochondrial encephalomyopathies: biochemical approach. (1660180)	DiMauro S.... Schon E.A.	1991	COX5A
36	Patients with idiopathic cardiomyopathy belong to the same mitochondrial DNA gene family of Parkinson's disease and mitochondrial encephalomyopathy. (2043137)	Ozawa T.... Hashiba K.	1991	MT-ND6
37	A mitochondrial encephalomyopathy due to partial cyt ochrome c oxidase deficiency with giant evoked potentials--a case report (2177689)	Higashi Y.... Shirabe T.	1990	DNAH8

Genes related to mitochondrial encephalomyopathy according to GeneCards:

(show top 50)    (show all 59)

id	Symbol	Description	Score	GeneCards Section Context
1	MELAS	mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes	11.1	Aliases & Descriptions
2	AIFM1	apoptosis-inducing factor, mitochondrion-associated, 1	11.1	Publications, Summaries
3	MT-ND5	mitochondrially encoded NADH dehydrogenase 5	11.0
4	MT-TK	mitochondrially encoded tRNA lysine	11.0	Publications
5	MT-TL1	mitochondrially encoded tRNA leucine 1 (UUA/G)	11.0	Publications
6	NDUFA1	NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa	11.0	Publications
7	MSTN	myostatin	11.0	Publications
8	GHRH	growth hormone releasing hormone	11.0	Publications
9	MT-ATP6	mitochondrially encoded ATP synthase 6	11.0
10	COX5A	cytochrome c oxidase subunit Va	11.0	Publications
11	CPOX	coproporphyrinogen oxidase	11.0	Publications
12	NDUFS4	NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase)	11.0	Publications
13	HSPD1	heat shock 60kDa protein 1 (chaperonin)	11.0	Publications
14	RYR1	ryanodine receptor 1 (skeletal)	11.0	Publications
15	MT-ND6	mitochondrially encoded NADH dehydrogenase 6	11.0	Publications
16	SOD2	superoxide dismutase 2, mitochondrial	11.0	Publications
17	MT-ND4	mitochondrially encoded NADH dehydrogenase 4	11.0
18	MT-ND1	mitochondrially encoded NADH dehydrogenase 1	11.0
19	CYCS	cytochrome c, somatic	11.0	Publications
20	GH1	growth hormone 1	11.0	Publications
21	MT-CO2	mitochondrially encoded cytochrome c oxidase II	11.0	Publications
22	COQ2	coenzyme Q2 homolog, prenyltransferase (yeast)	11.0	Summaries
23	MT-TR	mitochondrially encoded tRNA arginine	11.0	Publications
24	MT-TE	mitochondrially encoded tRNA glutamic acid	11.0	Publications
25	BCS1L	BCS1-like (S. cerevisiae)	11.0	Publications
26	FASTKD3	FAST kinase domains 3	11.0
27	NDUFA8	NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 8, 19kDa	11.0
28	NPTX2	neuronal pentraxin II	11.0
29	NPS	neuropeptide S	11.0
30	NDUFAF4	NADH dehydrogenase (ubiquinone) complex I, assembly factor 4	11.0
31	TRMU	tRNA 5-methylaminomethyl-2-thiouridylate methyltransferase	11.0
32	SURF1	surfeit 1	11.0	Publications
33	MT-CYB	mitochondrially encoded cytochrome b	11.0	Publications
34	TSFM	Ts translation elongation factor, mitochondrial	11.0
35	MRPS16	mitochondrial ribosomal protein S16	11.0
36	FASTKD2	FAST kinase domains 2	11.0	Publications
37	DARS2	aspartyl-tRNA synthetase 2, mitochondrial	11.0
38	FASTKD1	FAST kinase domains 1	11.0	Disorders
39	TFAM	transcription factor A, mitochondrial	11.0	Publications
40	FH	fumarate hydratase	11.0
41	LARS2	leucyl-tRNA synthetase 2, mitochondrial	11.0	Publications
42	CS	citrate synthase	11.0	Publications
43	BLVRB	biliverdin reductase B (flavin reductase (NADPH))	11.0	Publications
44	ELOVL7	ELOVL fatty acid elongase 7	11.0
45	SUCLA2	succinate-CoA ligase, ADP-forming, beta subunit	11.0	Publications
46	TK2	thymidine kinase 2, mitochondrial	11.0
47	CRAT	carnitine O-acetyltransferase	11.0	Publications
48	RARS2	arginyl-tRNA synthetase 2, mitochondrial	11.0
49	POLG	polymerase (DNA directed), gamma	11.0
50	NDUFAF2	NADH dehydrogenase (ubiquinone) complex I, assembly factor 2	11.0

Pathways related to mitochondrial encephalomyopathy according to GeneDecks:

id	Pathway	Score	Top Affiliating Genes
1	Citric acid cycle (TCA cycle)38	9.9	FH, SUCLA2, CS
2	Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins.38	9.2	NDUFS4, MT-CO1, MT-CO2, MT-ND4, MT-ATP6, MT-CYB
3	Oxidative phosphorylation20	9.1	MT-ND6, COX5A, NDUFA1, NDUFA8, NDUFS4, MT-ND1
4	Parkinsons disease20	9.1	NDUFS4, NDUFA8, MT-ND5, MT-CO1, MT-CO2, MT-ND4
5	Alzheimers disease20	8.9	NDUFS4, NDUFA8, NDUFA1, COX5A, CYCS, MT-CYB
6	Huntingtons disease20	8.6	NDUFA8, NDUFS4, TFAM, SOD1, SOD2, NDUFA1
7	Aminoacyl-tRNA biosynthesis20	8.6	DARS2, FARS2, LARS2, MT-TL1, MT-TK, MT-TR
8	Metabolic pathways20	7.1	COX5A, COQ2, NDUFA1, NDUFA8, NDUFS4, TK2

Compounds related to mitochondrial encephalomyopathy according to GeneDecks:

(show top 50)    (show all 161)

id	Compound	Score	Top Affiliating Genes
1	myxothiazol32 9 9	12.1	SOD2, NDUFS4, CYCS, MT-CYB
2	n acetylcysteine32	10.0	RPS27A, HSPD1, CRAT
3	ascorbic acid32 18	10.9	POMC, CS, MT-CYB, SOD2, COX5A, AIFM1
4	nitric oxide32 9 18 9	12.9	COX5A, CPOX, MT-CYB, BLVRB, CS
5	nad+32	9.7	NDUFS4, CS, RYR1, FH, AIFM1, MT-CYB
6	citrinin32	9.5	CAT, SOD1, NDUFS4, CYCS, MT-CYB
7	haem32	9.4	MT-CYB, CYCS, COX5A, MB, CPOX
8	pyruvate32	9.4	FH, HSPD1, SURF1, CRAT, MT-ND1, TFAM
9	antimycin a32	9.4	SOD2, MT-CYB, CAT, CYCS, NDUFS4, SOD1
10	glucose32	9.3	AIFM1, RYR1, MSTN, FH, MT-ND1, MT-CYB
11	fatty acid32	9.2	CRAT, GHRH, HSPD1, FH, TFAM, CPOX
12	trolox32	9.2	SOD1, MB, CAT, COX5A, CYCS, SOD2
13	malate32	9.2	DNAH8, SOD2, RPS27A, COX5A, NDUFS4, HSPD1
14	ubiquinone32	9.2	CAT, CYCS, NDUFS4, MT-ND4, COQ2, SOD1
15	fenton32	9.1	CP, MT-CYB, HSPD1, CAT, SOD1, SOD2
16	diphenyleneiodonium32	9.1	SOD1, CAT, CYCS, COX5A, NDUFS4, SOD2
17	hydroquinone32 18	10.0	CAT, CYCS, SOD1, SOD2, MT-CYB
18	alanine32	9.0	HSPD1, RYR1, POMC, DNAH8, CPOX, POLG
19	valine32	9.0	RPS27A, SOD2, MT-ND4, MB, MT-ND6, MT-ND1
20	dmpo32	9.0	SOD2, MB, CAT, SOD1, CP
21	diethyl dithiocarbamate32	9.0	POLG, SOD2, SOD1, TFAM, COX5A, CAT
22	peroxynitrite32	8.9	SOD2, CS, CPOX, MB, CAT, CYCS
23	dicoumarol32	8.9	RPS27A, SOD2, SOD1, CYCS, CAT
24	glutamate32	8.9	CP, NPTX2, CS, NDUFS4, COX5A, AIFM1
25	azide32	8.8	CP, MB, CAT, SOD1, RYR1
26	sodium azide32	8.8	SOD1, COX5A, DNAH8, CP, CAT
27	citrate32	8.5	SOD2, TFAM, NDUFS4, COX5A, CRAT, CAT
28	levodopa32 9 9	10.4	GHRH, CP, CS, SOD1, NDUFS4, RPS27A
29	Heme9 18 9	10.4	MB, CPOX, CAT, COX5A, CYCS, SUCLA2
30	rotenone32	8.3	CS, NDUFS4, MT-ND6, MB, CAT, AIFM1
31	copper32 18	9.3	CP, MT-CO2, MT-CO1, MT-CYB, HSPD1, CAT
32	superoxide32 18	9.2	MT-CYB, CAT, BLVRB, SOD1, NDUFS4, CS
33	sodium nitroprusside32	8.2	DNAH8, GH1, CP, SOD2, SOD1, COX5A
34	acetylcholine32 9 18 9	11.1	COX5A, GH1, CRAT, RYR1, BLVRB, POMC
35	doxorubicin32 34 9 9	11.1	DNAH8, RYR1, CRAT, NDUFS4, SOD2, RPS27A
36	4-hydroxynonenal32 18	9.1	CYCS, RPS27A, DNAH8, SOD2, CS, MB
37	hydrogen32 18	9.0	MT-CYB, RYR1, CS, DNAH8, NDUFS4, RPS27A
38	succinate32	8.0	RYR1, CPOX, CS, MB, FH, MT-CYB
39	creatinine32	7.9	MT-CYB, MB, HSPD1, GH1, RPS27A, COX5A
40	oxygen32 18	8.8	FH, MB, HSPD1, AIFM1, SURF1, CYCS
41	glutamine32	7.7	CP, RYR1, POMC, POLG, MB, HSPD1
42	aspartate32	7.7	CS, MB, CP, POMC, RPS27A, SOD2
43	indomethacin32 9 9	9.6	CAT, BLVRB, HSPD1, RPS27A, COX5A, DNAH8
44	adenylate32	7.5	GH1, BLVRB, DNAH8, RPS27A, CRAT, AIFM1
45	h2o232	7.2	NDUFS4, SOD1, RYR1, SOD2, MT-CYB, MB
46	arginine32	7.2	POMC, GHRH, SOD2, SOD1, RYR1, DNAH8
47	iron32 18	7.9	CS, SOD2, SOD1, CPOX, CP, NDUFS4
48	atp32	6.7	MT-ND5, POLG, CPOX, BLVRB, CS, SOD2
49	nadh32 9 18 9	9.6	BLVRB, MT-ND5, MT-CO1, MT-CO2, MT-ND4, MT-ATP6
50	lactate32	6.5	MT-ND4, MT-ND1, FH, MB, HSPD1, CAT

Cellular components related to mitochondrial encephalomyopathy according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	mitochondrial intermembrane space	GO:005758	9.5	CAT, AIFM1, CYCS, NDUFA8, CPOX
2	mitochondrial inner membrane	GO:005743	9.3	CPOX, NDUFS4, NDUFA8, NDUFA1, COX5A, CRAT
3	mitochondrial matrix	GO:005759	7.3	TK2, TFAM, SOD1, SOD2, CS, DARS2
4	mitochondrion	GO:005739	6.8	BCS1L, NDUFA8, NDUFAF2, NDUFAF4, NDUFS4, TSFM

Biological processes related to mitochondrial encephalomyopathy according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	mitochondrial respiratory chain complex I assembly	GO:032981	10.2	NDUFS4, NDUFAF4, AIFM1, BCS1L
2	cellular respiration	GO:045333	10.0	FASTKD3, FASTKD2, FASTKD1, CYCS, NDUFS4
3	respiratory electron transport chain	GO:022904	10.0	NDUFS4, NDUFA8, NDUFA1, COX5A, CYCS
4	apoptotic DNA fragmentation	GO:006309	9.9	AIFM1, CYCS, SOD1
5	tRNA aminoacylation for protein translation	GO:006418	8.7	RARS2, LARS2, FARS2, DARS2
6	small molecule metabolic process	GO:044281	7.5	CPOX, POMC, CS, TK2, ELOVL7, NDUFS4

Molecular functions related to mitochondrial encephalomyopathy according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	superoxide dismutase activity	GO:004784	10.0	SOD1, SOD2
2	NADH dehydrogenase (ubiquinone) activity	GO:008137	9.6	NDUFA1, NDUFA8, NDUFAF2, NDUFS4