MCID: MTR014
MIFTS: 62

Motor Neuron Disease malady

Neuronal diseases category
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Summaries for Motor Neuron Disease

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NINDS:43 The motor neuron diseases (MNDs) are a group of progressive neurological disorders that destroy cells that control essential muscle activity such as speaking, walking, breathing, and swallowing.

MalaCards based summary: Motor Neuron Disease, also known as motor neuron diseases, is related to lateral sclerosis and progressive muscular atrophy. An important gene associated with Motor Neuron Disease is SOD2 (superoxide dismutase 2, mitochondrial), and among its related pathways are Oxidative Stress and superoxide radicals degradation. The compounds mntmpyp and pyrogallol have been mentioned in the context of this disorder. Affiliated tissues include the motor neurones, spinal cord and brain, and related mouse phenotypes are hearing/vestibular/ear and adipose tissue.

Disease Ontology:8 A neurodegenerative disease that is located in the motor neurones.

Wikipedia:65 Motor neuron disease (MND) are a small group of neurological disorders that selectively affect motor... more...

Aliases & Classifications for Motor Neuron Disease

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Motor Neuron Disease, Aliases & Descriptions:

Name: Motor Neuron Disease 30 8 10 44 62
 
Motor Neuron Diseases 43 62


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Neuronal diseases


External Ids:

Disease Ontology8 DOID:231
MeSH34 D016472
ICD9CM27 335.2
ICD1025 G12.2

Related Diseases for Motor Neuron Disease

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Diseases related to Motor Neuron Disease via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 143)
idRelated DiseaseScoreTop Affiliating Genes
1lateral sclerosis31.8TARDBP
2progressive muscular atrophy31.1SMN1
3spinal muscular atrophy31.1SMN1, SMN2
4neuropathy31.0SMN1, SMN2, NEFH, SOD2
5frontotemporal dementia31.0TARDBP, RPS27A, SOD1
6muscular atrophy30.7NAIP, SMN2, SMN1
7semantic dementia30.7RPS27A
8progressive supranuclear palsy30.6TARDBP
9huntington's disease30.4CNTF
10parkinson's disease30.4SOD2, SOD1, RPS27A, CNTF
11pick's disease30.1TARDBP, RPS27A
12amyotrophic lateral sclerosis type 1428.8SMN1, SMN2, SOD2, SOD1, NAIP, TARDBP
13alzheimer's disease28.8NAIP, NEFH, RPS27A, SOD2, CNTF, SOD1
14neuronitis11.4
15amyotrophic lateral sclerosis10.9
16dementia10.9
17madras motor neuron disease10.6
18cervicitis10.4
19gangliosidosis10.4
20spasticity10.4
21ataxia10.3
22multiple sclerosis10.3
23sandhoff disease10.3
24tauopathy10.3
25chorea10.3
26alcoholic liver cirrhosis10.3SOD2, SOD1
27multifocal motor neuropathy10.2
28brown-vialetto-van laere syndrome10.2
29kennedy's disease10.2
30cerebritis10.2
31hyperparathyroidism10.2
32spinocerebellar degeneration10.2
33monomelic amyotrophy10.2
34benign focal amyotrophy10.2
35schimke immunoosseous dysplasia10.2SMN1, SMN2
36tay-sachs disease10.2SMN1, HEXB
37psychotic disorder10.2SOD2, CNTF
38diabetic retinopathy10.2SOD2, SOD1
39spondylosis10.1
40progressive bulbar palsy10.1
41werdnig-hoffmann disease10.1
42amyotrophic lateral sclerosis type 210.1
43ftdals110.1
44adenoma10.1
45apraxia10.1
46breast cancer10.1
47myositis10.1
48peripheral neuropathy10.1
49polyneuropathy10.1
50spinocerebellar ataxia10.1

Graphical network of the top 20 diseases related to Motor Neuron Disease:



Diseases related to motor neuron disease

Symptoms for Motor Neuron Disease

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Drugs & Therapeutics for Motor Neuron Disease

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Drug clinical trials:

Search ClinicalTrials for Motor Neuron Disease

Search NIH Clinical Center for Motor Neuron Disease

Cell-based therapeutics:


LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine
Read about Motor Neuron Disease cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Motor Neuron Disease:
Embryonic stem cell-derived motor neuron transplantation to treat paralysis
Embryonic/Adult Cultured Cells Related to Motor Neuron Disease:
Neuronal progenitor cells

Genetic Tests for Motor Neuron Disease

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Anatomical Context for Motor Neuron Disease

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MalaCards organs/tissues related to Motor Neuron Disease:

32
Spinal cord, Brain, Cortex, Skeletal muscle, Breast, Testes, T cells, Eye, Bone, Thyroid, Monocytes, B cells, Temporal lobe, Tongue

FMA organs/tissues related to Motor Neuron Disease:

14
The motor neurones

LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine

Cells/anatomical compartments in embryo or adult related to Motor Neuron Disease:
id TissueAnatomical CompartmentCell Relevance
1 Neural TubeMotor Neural Progenitor DomainMotor Neural Progenitor Cells Potential therapeutic candidate
2 Neural TubeMotor Neural Progenitor DomainMotor Neurons Potential therapeutic candidate, affected by disease

Animal Models for Motor Neuron Disease or affiliated genes

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MGI Mouse Phenotypes related to Motor Neuron Disease:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053778.7SMN1, SOD2, SOD1, HEXB
2MP:00053758.4CTF1, TARDBP, HEXB, SOD2
3MP:00053697.8SMN1, SOD2, SOD1, HEXB, CNTF, TARDBP
4MP:00053867.1SMN1, SOD2, SOD1, HEXB, NEFH, CNTF
5MP:00053766.8SMN1, SOD2, SOD1, NAIP, HEXB, CNTF
6MP:00053846.5CTF1, SMN1, SOD2, SOD1, NAIP, HEXB
7MP:00036316.5SMN1, SOD2, SOD1, NAIP, HEXB, NEFH

Publications for Motor Neuron Disease

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Articles related to Motor Neuron Disease:

(show top 50)    (show all 638)
idTitleAuthorsYear
1
Motor neuron disease: A large case-control study finds no association between living near power lines and risk of ALS. (25330726)
2014
2
The personal experience of partners of individuals with motor neuron disease. (22973854)
2013
3
The relationship between motor neuron disease and bullous pemphigoid: an English cohort study. (24124826)
2013
4
Orofacial apraxia in motor neuron disease. (23569452)
2013
5
Neurogenic and myogenic contributions to hereditary motor neuron disease. (22327341)
2012
6
Exome sequencing reveals riboflavin transporter mutations as a cause of motor neuron disease. (22740598)
2012
7
Motor neuron disease: common conformational change identified in toxic SOD1 mutants-a novel diagnostic marker for ALS? (22777249)
2012
8
Occurrence of eye movement disorders in motor neuron disease. (22117133)
2012
9
Motor neuron disease: the C9orf72 hexanucleotide repeat expansion in FTD and ALS. (22487746)
2012
10
The impact of perceived lack of support provided by health and social care services to caregivers of people with motor neuron disease. (22292844)
2012
11
Diaphragm pacing and noninvasive respiratory management of amyotrophic lateral sclerosis/motor neuron disease. (23042087)
2012
12
Cognitive dysfunction in lower motor neuron disease: executive and memory deficits in progressive muscular atrophy. (20562407)
2011
13
Trends in death certification for multiple sclerosis, motor neuron disease, Parkinson's disease and epilepsy in English populations 1979-2006. (19946783)
2010
14
Motor neuron disease: systematic reviews of treatment for ALS and SMA. (20015852)
2010
15
Motor neuron disease: focusing the mind on ALS: updated practice parameters. (20379204)
2010
16
Survival profiles of patients with frontotemporal dementia and motor neuron disease. (19901167)
2009
17
Motor neuron disease. (19131860)
2009
18
Machado-Joseph disease presenting as motor neuron disease. (17963092)
2008
19
Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease. (18425913)
2008
20
Re: Brain SPECT perfusion of frontotemporal dementia associated with motor neuron disease. (18427076)
2008
21
Gene expression analysis of frontotemporal lobar degeneration of the motor neuron disease type with ubiquitinated inclusions. (17569064)
2007
22
Elevated levels of transcripts encoding a human retroviral envelope protein (syncytin) in muscles from patients with motor neuron disease. (17453631)
2007
23
Brain spect perfusion of frontotemporal dementia associated with motor neuron disease. (17664410)
2007
24
Motor neuron disease mortality in Great Britain continues to rise: examination of mortality rates 1975 - 2004. (18033591)
2007
25
Low yield in screening patients with sporadic motor neuron disease for Kennedy disease. (17908088)
2007
26
Detoxication gene polymorphism and susceptibility to sporadic motor neuron disease in Russian population]. (16459354)
2006
27
Treatment for spasticity in amyotrophic lateral sclerosis/motor neuron disease. (16437474)
2006
28
Role of neurofilament aggregation in motor neuron disease. (17006927)
2006
29
How does current care practice influence the experience of a new diagnosis of motor neuron disease? A qualitative study of current guidelines-based practice. (16963405)
2006
30
Cytoplasmic body myopathy masquerading as motor neuron disease. (15389660)
2004
31
Riluzole slows the progression of neuromuscular dysfunction in the wobbler mouse motor neuron disease. (15306257)
2004
32
Occupational exposure to metals and solvents and the risk of motor neuron disease. A case-control study. (14557686)
2003
33
Cerebral amyloid angiopathy and motor neurone disease presenting with a progressive supranuclear palsy-like syndrome. (12621639)
2003
34
Increased expression level of the splicing variant of SIP1 in motor neuron diseases. (11943600)
2002
35
Reduced endplate currents underlie motor unit dysfunction in canine motor neuron disease. (12466447)
2002
36
Elevation of the soft palate in speech and swallowing in normal female participants and females with motor neuron disease: an innovative procedure for measuring palatal elevation. (12012616)
2002
37
Dementia with motor neuron disease. (11037194)
2000
38
Treatable lower motor neuron disease due to vitamin D deficiency and secondary hyperparathyroidism. (11465022)
2000
39
Striatonigral degeneration with motor neuron disease. (10896276)
2000
40
Motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration. (10435507)
1999
41
Troyer syndrome: a combination of central brain abnormality and motor neuron disease? (10463356)
1999
42
Localization and expression of ciliary neurotrophic factor (CNTF) in postmortem sciatic nerve from patients with motor neuron disease and diabetic neuropathy. (8759781)
1996
43
Decreased synaptophysin immunoreactivity of the anterior horns in motor neuron disease. (8171961)
1994
44
Parvalbumin and calbindin D-28k in the human motor system and in motor neuron disease. (8232749)
1993
45
Kennedy's disease: genetic diagnosis of an inherited form of motor neuron disease. (8517843)
1993
46
Characterisation of the distribution of choline acetyltransferase messenger RNA in human spinal cord and its depletion in motor neurone disease. (1469422)
1992
47
Antibodies to the ganglioside GD1b in a patient with motor neuron disease and thyroid adenoma. (1953407)
1991
48
Neurophysiological study in chronic GM2 gangliosidosis (hexosaminidase A and B deficiency), with motor neuron disease phenotype. (2793417)
1989
49
Myokymia of the tongue in a case of brainstem tumour that simulated motor-neuron disease. (3185343)
1988
50
F-response frequency in motor neuron disease and cervical spondylosis. (3585384)
1987

Variations for Motor Neuron Disease

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Expression for genes affiliated with Motor Neuron Disease

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Expression patterns in normal tissues for genes affiliated with Motor Neuron Disease

Search GEO for disease gene expression data for Motor Neuron Disease.

Pathways for genes affiliated with Motor Neuron Disease

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Pathways related to Motor Neuron Disease according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.9SOD1, SOD2
29.9SOD1, SOD2
3
Show member pathways
9.9SOD1, SOD2
4
Show member pathways
Amyotrophic lateral sclerosis (ALS)37
9.8NEFH, SOD1
5
Show member pathways
9.4SOD2, SOD1, RPS27A
68.9TARDBP, NEFH, SOD1

Compounds for genes affiliated with Motor Neuron Disease

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Compounds related to Motor Neuron Disease according to GeneCards/GeneDecks:

(show top 50)    (show all 56)
idCompoundScoreTop Affiliating Genes
1mntmpyp4410.3SOD2, SOD1
2pyrogallol4410.3SOD2, SOD1
3zinc superoxide4410.3SOD2, SOD1
4propyl gallate4410.3SOD1, SOD2
5dmpo4410.3SOD2, SOD1
63-nitrotyrosine4410.3SOD1, SOD2
7glutathion4410.3SOD2, SOD1
83-amino-1,2,4-triazole4410.3SOD1, SOD2
9nsc344410.3SOD1, SMN2
10onoo4410.3SOD1, SOD2
11fenton4410.3SOD2, SOD1
12antimycin a4410.3SOD1, SOD2
13dehydroascorbic acid44 1111.3SOD2, SOD1
14trolox4410.2SOD1, SOD2
158-oxo-dg4410.2SOD1, SOD2
16allopurinol44 50 1112.2SOD2, SOD1
17hydroquinone44 2411.2SOD2, SOD1
18selegiline44 1111.2SOD1, SOD2
19aclarubicin4410.2SMN1, SMN2
20tocopherol4410.2SOD1, SOD2
21selenite4410.2SOD1, SOD2
22gssg4410.2SOD2, SOD1
23phenylbutyrate4410.2SMN2, SMN1
24diethyl dithiocarbamate4410.1SOD1, SOD2
25Hydrogen peroxide2410.1SOD1, SOD2
26il 114410.1CNTF, CTF1
27nitroglycerin44 1111.0SOD1, SOD2
28ndga4410.0SOD1, SOD2
29hypoxanthine44 24 1111.9SOD1, SOD2
30diphenyleneiodonium449.9SOD1, SOD2
31formate449.9RPS27A, SOD1
32dicoumarol449.8RPS27A, SOD1, SOD2
33rotenone449.8RPS27A, SOD1, SOD2
346-hydroxydopamine449.8RPS27A, SOD1, SOD2
35ascorbic acid44 2410.8CNTF, SOD1, SOD2
364-hydroxynonenal44 2410.8RPS27A, SOD1, SOD2
37methamphetamine44 50 1111.7SOD2, RPS27A, CNTF
38guanidine hydrochloride449.7CNTF, RPS27A, SOD2
39indomethacin44 28 61 1112.6RPS27A, SOD1, SOD2
40butyrate449.4RPS27A, SOD2, SMN2, SMN1
41n acetylcysteine449.4SOD2, SOD1, RPS27A, CNTF
42acetylcholine44 50 28 24 1113.4SOD2, RPS27A, CNTF
43h2o2449.4CNTF, RPS27A, SOD1, SOD2
44alanine449.3SOD2, SOD1, RPS27A, CNTF
45actinomycin d449.2SOD2, RPS27A, CNTF
46cisplatin44 50 61 1112.1SOD2, SOD1, NAIP, RPS27A
47arginine449.1SMN1, SOD2, SOD1, RPS27A, CNTF
48tyrosine448.8SMN1, SOD2, NEFH, RPS27A, CNTF
49superoxide44 249.7TARDBP, CNTF, NEFH, SOD1, SOD2
50cysteine448.4RPS27A, NAIP, SOD1, SOD2

GO Terms for genes affiliated with Motor Neuron Disease

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Biological processes related to Motor Neuron Disease according to GeneCards/GeneDecks:

(show all 15)
idNameGO IDScoreTop Affiliating Genes
1response to superoxideGO:00030310.1SOD1, SOD2
2hydrogen peroxide biosynthetic processGO:05066510.1SOD1, SOD2
3removal of superoxide radicalsGO:01943010.1SOD2, SOD1
4superoxide anion generationGO:04255410.1SOD1, SOD2
5superoxide metabolic processGO:00680110.0SOD1, SOD2
6regulation of mitochondrial membrane potentialGO:05188110.0SOD2, SOD1
7response to axon injuryGO:04867810.0SOD1, SOD2
8regulation of blood pressureGO:0082179.9SOD1, SOD2
9glutathione metabolic processGO:0067499.8SOD1, SOD2
10neuron developmentGO:0486669.6CTF1, CNTF, SOD2
11locomotory behaviorGO:0076269.6SOD2, SOD1, HEXB
12response to hydrogen peroxideGO:0425429.6SOD1, SOD2
13negative regulation of neuron apoptotic processGO:0435249.2CNTF, NAIP, SOD1, SOD2
14nervous system developmentGO:0073999.1SMN2, NAIP, CTF1
15cell deathGO:0082198.6SMN2, HEXB, NEFH, TARDBP

Molecular functions related to Motor Neuron Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1superoxide dismutase activityGO:0047849.6SOD1, SOD2
2identical protein bindingGO:0428028.7TARDBP, SOD1, SOD2, SMN2

Products for genes affiliated with Motor Neuron Disease

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Sources for Motor Neuron Disease

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet