MCID: MTR014
MIFTS: 61

Motor Neuron Disease malady

Neuronal diseases category

Summaries for Motor Neuron Disease

About this section
Sources:
9Disease Ontology, 45NINDS, 66Wikipedia, 34MalaCards
See all sources

Fully expand this MalaCard

Download this MalaCard
NINDS:45 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: Motor Neuron Disease, also known as motor neuron diseases, is related to lateral sclerosis and frontotemporal dementia. 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:9 A neurodegenerative disease that is located in the motor neurones.

Wikipedia:66 The motor neuron diseases (MND) are a group of neurological disorders that selectively affect motor... more...

Aliases & Classifications for Motor Neuron Disease

About this section
Sources:
9Disease Ontology, 11DISEASES, 46Novoseek, 32LifeMap Discoveryā„¢, 63UMLS, 45NINDS, 59SNOMED-CT, 28ICD9CM, 36MeSH, 26ICD10
See all sources

Classifications:

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


Aliases & Descriptions:

motor neuron disease 9 11 46 32 63
motor neuron diseases 45


External Ids:

Disease Ontology9 DOID:231
ICD9CM28 335.2
MeSH36 D016472
ICD1026 G12.2

Related Diseases for Motor Neuron Disease

About this section
Sources:
18GeneCards, 19GeneDecks
See all sources

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

(show top 50)    (show all 140)
idRelated DiseaseScoreTop Affiliating Genes
1lateral sclerosis31.5TARDBP
2frontotemporal dementia31.1TARDBP, RPS27A, SOD1
3amyotrophic lateral sclerosis31.0SMN1, SMN2, SOD2, SOD1, NAIP, NEFH
4neuropathy30.9SMN1, SMN2, SOD2, NEFH
5muscular atrophy30.6SMN1, SMN2, NAIP
6spinal muscular atrophy30.6SMN2, SMN1
7progressive muscular atrophy30.6SMN1
8parkinson's disease30.5CNTF, RPS27A, SOD1, SOD2
9progressive supranuclear palsy30.4TARDBP
10semantic dementia30.3RPS27A
11pick's disease30.2TARDBP, RPS27A
12huntington's disease30.0CNTF
13alzheimer's disease29.7SOD2, SOD1, NAIP, NEFH, RPS27A, CNTF
14neuronitis11.4
15dementia10.9
16madras motor neuron disease10.6
17cervicitis10.4
18gangliosidosis10.4
19spasticity10.4
20ataxia10.3
21tauopathy10.3
22sandhoff disease10.3
23multiple sclerosis10.3
24chorea10.3
25kennedy's disease10.2
26cerebritis10.2
27brown-vialetto-van laere syndrome10.2
28hyperparathyroidism10.2
29spinocerebellar degeneration10.2
30monomelic amyotrophy10.2
31multifocal motor neuropathy10.2
32benign focal amyotrophy10.2
33spondylosis10.1
34progressive bulbar palsy10.1
35distal hereditary motor neuropathy10.1
36anterior horn cell disease10.1
37werdnig-hoffmann disease10.1
38adenoma10.1
39apraxia10.1
40myositis10.1
41peripheral neuropathy10.1
42polyneuropathy10.1
43spinocerebellar ataxia10.1
44infantile-onset ascending hereditary spastic paralysis10.1
45acanthocytosis10.1
46lathyrism10.1
47amyotrophic lateral sclerosis with frontotemporal dementia10.1
48spinal muscular atrophy, distal, autosomal recessive, 410.1
49myokymia10.1
50alcoholic liver cirrhosis10.1SOD1, SOD2

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



Diseases related to motor neuron disease

Symptoms for Motor Neuron Disease

About this section

Drugs & Therapeutics for Motor Neuron Disease

About this section
Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 32LifeMap Discoveryā„¢, 63UMLS, 42NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Motor Neuron Disease

Drug clinical trials:

Search ClinicalTrials for Motor Neuron Disease

Search NIH Clinical Center for Motor Neuron Disease

Search CenterWatch 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

About this section

Anatomical Context for Motor Neuron Disease

About this section
Sources:
32LifeMap Discoveryā„¢, 34MalaCards, 15FMA
See all sources

MalaCards organs/tissues related to Motor Neuron Disease:

34
Spinal cord, Brain, Cortex, Skeletal muscle, Testes, Tongue, Temporal lobe, B cells, Monocytes, Breast, Thyroid, Bone, Eye, T cells

FMA organs/tissues related to Motor Neuron Disease:

15
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

About this section
Sources:
38MGI
See all sources

MGI Mouse Phenotypes related to Motor Neuron Disease:

38
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

About this section
Sources:
53PubMed
See all sources

Articles related to Motor Neuron Disease:

(show top 50)    (show all 614)
idTitleAuthorsYear
1
The personal experience of partners of individuals with motor neuron disease. (22973854)
2013
2
The relationship between motor neuron disease and bullous pemphigoid: an English cohort study. (24124826)
2013
3
Orofacial apraxia in motor neuron disease. (23569452)
2013
4
Neurogenic and myogenic contributions to hereditary motor neuron disease. (22327341)
2012
5
Exome sequencing reveals riboflavin transporter mutations as a cause of motor neuron disease. (22740598)
2012
6
Motor neuron disease: common conformational change identified in toxic SOD1 mutants-a novel diagnostic marker for ALS? (22777249)
2012
7
Occurrence of eye movement disorders in motor neuron disease. (22117133)
2012
8
Motor neuron disease: the C9orf72 hexanucleotide repeat expansion in FTD and ALS. (22487746)
2012
9
The impact of perceived lack of support provided by health and social care services to caregivers of people with motor neuron disease. (22292844)
2012
10
Diaphragm pacing and noninvasive respiratory management of amyotrophic lateral sclerosis/motor neuron disease. (23042087)
2012
11
Cognitive dysfunction in lower motor neuron disease: executive and memory deficits in progressive muscular atrophy. (20562407)
2011
12
Trends in death certification for multiple sclerosis, motor neuron disease, Parkinson's disease and epilepsy in English populations 1979-2006. (19946783)
2010
13
Motor neuron disease: systematic reviews of treatment for ALS and SMA. (20015852)
2010
14
Motor neuron disease: focusing the mind on ALS: updated practice parameters. (20379204)
2010
15
Survival profiles of patients with frontotemporal dementia and motor neuron disease. (19901167)
2009
16
Motor neuron disease. (19131860)
2009
17
Machado-Joseph disease presenting as motor neuron disease. (17963092)
2008
18
Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease. (18425913)
2008
19
Re: Brain SPECT perfusion of frontotemporal dementia associated with motor neuron disease. (18427076)
2008
20
Gene expression analysis of frontotemporal lobar degeneration of the motor neuron disease type with ubiquitinated inclusions. (17569064)
2007
21
Elevated levels of transcripts encoding a human retroviral envelope protein (syncytin) in muscles from patients with motor neuron disease. (17453631)
2007
22
Brain spect perfusion of frontotemporal dementia associated with motor neuron disease. (17664410)
2007
23
Motor neuron disease mortality in Great Britain continues to rise: examination of mortality rates 1975 - 2004. (18033591)
2007
24
Low yield in screening patients with sporadic motor neuron disease for Kennedy disease. (17908088)
2007
25
Nogo-A marks motor neuron disease. (17474106)
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
Analysis of the glutathione S-transferase P1 gene Ile105Val polymorphism in the patients with sporadic motor neuron disease from Russia]. (15341277)
2004
31
Cytoplasmic body myopathy masquerading as motor neuron disease. (15389660)
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

About this section

Expression for genes affiliated with Motor Neuron Disease

About this section
Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
See all sources
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

About this section
Sources:
51PathCards, 39NCBI BioSystems Database, 56Reactome, 52PharmGKB, 31KEGG, 54QIAGEN, 5Cell Signaling Technology
See all sources

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)39
9.8NEFH, SOD1
5
Show member pathways
9.4SOD2, SOD1, RPS27A
68.9TARDBP, NEFH, SOD1

Compounds for genes affiliated with Motor Neuron Disease

About this section
Sources:
46Novoseek, 12DrugBank, 52PharmGKB, 25HMDB, 30IUPHAR, 62Tocris Bioscience
See all sources

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

(show top 50)    (show all 56)
idCompoundScoreTop Affiliating Genes
1mntmpyp4610.3SOD2, SOD1
2pyrogallol4610.3SOD2, SOD1
3zinc superoxide4610.3SOD2, SOD1
4propyl gallate4610.3SOD1, SOD2
5dmpo4610.3SOD2, SOD1
63-nitrotyrosine4610.3SOD1, SOD2
7glutathion4610.3SOD2, SOD1
83-amino-1,2,4-triazole4610.3SOD1, SOD2
9nsc344610.3SOD1, SMN2
10onoo4610.3SOD1, SOD2
11fenton4610.3SOD2, SOD1
12antimycin a4610.3SOD1, SOD2
13dehydroascorbic acid46 1211.3SOD2, SOD1
14trolox4610.2SOD1, SOD2
158-oxo-dg4610.2SOD1, SOD2
16allopurinol46 52 1212.2SOD2, SOD1
17hydroquinone46 2511.2SOD2, SOD1
18selegiline46 1211.2SOD1, SOD2
19aclarubicin4610.2SMN1, SMN2
20tocopherol4610.2SOD1, SOD2
21selenite4610.2SOD1, SOD2
22gssg4610.2SOD2, SOD1
23phenylbutyrate4610.2SMN2, SMN1
24diethyl dithiocarbamate4610.1SOD1, SOD2
25Hydrogen peroxide2510.1SOD1, SOD2
26il 114610.1CNTF, CTF1
27nitroglycerin46 1211.0SOD1, SOD2
28ndga4610.0SOD1, SOD2
29hypoxanthine46 25 1211.9SOD1, SOD2
30diphenyleneiodonium469.9SOD1, SOD2
31formate469.9RPS27A, SOD1
32dicoumarol469.8RPS27A, SOD1, SOD2
33rotenone469.8RPS27A, SOD1, SOD2
346-hydroxydopamine469.8RPS27A, SOD1, SOD2
35ascorbic acid46 2510.8CNTF, SOD1, SOD2
364-hydroxynonenal46 2510.8RPS27A, SOD1, SOD2
37methamphetamine46 52 1211.7SOD2, RPS27A, CNTF
38guanidine hydrochloride469.7CNTF, RPS27A, SOD2
39indomethacin46 30 62 1212.6RPS27A, SOD1, SOD2
40butyrate469.4RPS27A, SOD2, SMN2, SMN1
41n acetylcysteine469.4SOD2, SOD1, RPS27A, CNTF
42acetylcholine46 52 30 25 1213.4SOD2, RPS27A, CNTF
43h2o2469.4CNTF, RPS27A, SOD1, SOD2
44alanine469.3SOD2, SOD1, RPS27A, CNTF
45actinomycin d469.2SOD2, RPS27A, CNTF
46cisplatin46 52 62 1212.1SOD2, SOD1, NAIP, RPS27A
47arginine469.1SMN1, SOD2, SOD1, RPS27A, CNTF
48tyrosine468.8SMN1, SOD2, NEFH, RPS27A, CNTF
49superoxide46 259.7TARDBP, CNTF, NEFH, SOD1, SOD2
50cysteine468.4RPS27A, NAIP, SOD1, SOD2

GO Terms for genes affiliated with Motor Neuron Disease

About this section
Sources:
17Gene Ontology
See all sources

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

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Motor Neuron Disease

About this section
4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet