MCID: MTR014
MIFTS: 63

Motor Neuron Disease malady

Neuronal diseases category

Summaries for Motor Neuron Disease

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8Disease Ontology, 43NINDS, 63Wikipedia, 32MalaCards
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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: Motor Neuron Disease, also known as motor neuron diseases, is related to amyotrophic lateral sclerosis and lateral sclerosis. An important gene associated with Motor Neuron Disease is SOD2 (superoxide dismutase 2, mitochondrial), and among its related pathways are superoxide radicals degradation and Pathogenesis of ALS. The compounds 6-hydroxydopamine and superoxide have been mentioned in the context of this disorder. Affiliated tissues include the motor neurones, spinal cord and brain, and related mouse phenotypes are nervous system and behavior/neurological.

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

Wikipedia:63 The motor neuron diseases (MND) are a group of neurological disorders that selectively affect motor... more...

Aliases & Classifications for Motor Neuron Disease

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8Disease Ontology, 10DISEASES, 44Novoseek, 30LifeMap Discovery™, 60UMLS, 43NINDS, 27ICD9CM, 56SNOMED-CT, 34MeSH, 25ICD10
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Classifications:

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


Aliases & Descriptions:

motor neuron disease 8 10 44 30 60
motor neuron diseases 43


External Ids:

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

Related Diseases for Motor Neuron Disease

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Sources:
17GeneCards, 18GeneDecks
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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 167)
idRelated DiseaseScoreTop Affiliating Genes
1amyotrophic lateral sclerosis31.4DCTN1, NEFH
2lateral sclerosis31.4SOD1, ALS2
3dementia31.3RPS27A, PVALB, HEXA, SYP, TARDBP
4frontotemporal dementia31.1RPS27A, SOD1, INA, TARDBP
5neuropathy30.9SOD2, NEFH, SMN1, SMN2
6muscular atrophy30.6NAIP, SMN1, SMN2
7neuromuscular disease30.5DCTN1, GDNF, NAIP, SMN1, SMN2
8gangliosidosis30.5HEXB, HEXA
9spinal muscular atrophy30.5SMN2, SMN1
10parkinson's disease30.5GDNF, RPS27A
11multiple sclerosis30.4CNTF
12sandhoff disease30.4HEXA, HEXB
13progressive supranuclear palsy30.3TARDBP
14pick's disease30.2TARDBP, RPS27A
15polyneuropathy30.2GDNF, CNTF
16adenoma30.2GDNF, SYP
17schizophrenia30.0GDNF, CNTF, SYP, PVALB
18huntington's disease30.0RPS27A, SOD1, CNTF, GDNF
19myopathy30.0RPS27A, SMN1
20alzheimer's disease30.0NEFH, SOD1, SOD2, PVALB, RPS27A, SYP
21myeloma30.0CNTF
22diabetic neuropathy30.0GDNF, CNTF
23neuronitis11.4
24spinal cord disease10.6
25brain disease10.5
26madras motor neuron disease10.4
27cervicitis10.4
28gangliosidosis gm210.4
29progressive muscular atrophy10.3
30anterior horn cell disease10.3
31tauopathy10.3
32axonal neuropathy10.2
33cerebritis10.2
34brown-vialetto-van laere syndrome10.2
35kennedy's disease10.2
36hyperparathyroidism10.2
37nervous system disease10.2
38spinocerebellar degeneration10.2
39monomelic amyotrophy10.2
40benign focal amyotrophy10.2
41werdnig-hoffmann disease10.1
42distal hereditary motor neuropathy10.1
43striatonigral degeneration10.1
44spondylosis10.1
45monoclonal paraproteinemia10.1
46motor peripheral neuropathy10.1
47progressive bulbar palsy10.1
48apraxia10.1
49autosomal recessive disease10.1
50central nervous system disease10.1

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



Diseases related to motor neuron disease

Clinical Features for Motor Neuron Disease

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Drugs & Therapeutics for Motor Neuron Disease

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 30LifeMap Discovery™, 60UMLS, 40NDF-RT
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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

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Anatomical Context for Motor Neuron Disease

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Sources:
30LifeMap Discovery™, 32MalaCards, 14FMA
See all sources

MalaCards organs/tissues related to Motor Neuron Disease:

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

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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Sources:
36MGI
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MGI Mouse Phenotypes related to Motor Neuron Disease:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000363110.6ALS2, PVALB, HEXA, HEXB, SOD2, SOD1
2MP:000538610.6CTF1, SOD1, SOD2, HEXB, HEXA, PVALB
3MP:000536910.5PVALB, HEXB, SOD2, SOD1, CNTF, GDNF
4MP:000538410.5PVALB, HEXB, SOD2, SOD1, CTF1, BSCL2
5MP:000301210.4SMN1, TARDBP, CNTF, SYP, BSCL2, PVALB
6MP:000537610.4HEXA, HEXB, SOD2, SOD1, CTF1, BSCL2
7MP:000537710.4SMN1
8MP:000537510.2HEXB, SOD2, CTF1, BSCL2, TARDBP

Publications for Motor Neuron Disease

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Sources:
50PubMed
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Articles related to Motor Neuron Disease:

(show top 50)    (show all 658)
idTitleAuthorsYear
1
Blue-green algae or cyanobacteria in the intestinal micro-flora may produce neurotoxins such as Beta-N-Methylamino-L-Alanine (BMAA) which may be related to development of amyotrophic lateral sclerosis, Alzheimer's disease and Parkinson-Dementia-Complex in humans and Equine Motor Neuron Disease in horses. (23146671)
2013
2
Motor neuron disease: common conformational change identified in toxic SOD1 mutants-a novel diagnostic marker for ALS? (22777249)
2012
3
Occurrence of eye movement disorders in motor neuron disease. (22117133)
2012
4
Diaphragm pacing and noninvasive respiratory management of amyotrophic lateral sclerosis/motor neuron disease. (23042087)
2012
5
Treatment for cramps in amyotrophic lateral sclerosis/motor neuron disease. (22513921)
2012
6
What wires together dies together: verbs, actions and neurodegeneration in motor neuron disease. (21924711)
2012
7
Recombinant human insulin-like growth factor I (rhIGF-I) for the treatment of amyotrophic lateral sclerosis/motor neuron disease. (23152212)
2012
8
Motor neuron disease: urgently needed-biomarkers for amyotrophic lateral sclerosis. (21221113)
2011
9
Semantic dementia with lower motor neuron disease showing FTLD-TDP type 3 pathology (sensu Mackenzie). (21029204)
2011
10
Who will benefit from tracheostomy ventilation in motor neuron disease? (21813619)
2011
11
Globular glial tauopathies (GGT) presenting with motor neuron disease or frontotemporal dementia: an emerging group of 4-repeat tauopathies. (21773886)
2011
12
Enteral tube feeding for amyotrophic lateral sclerosis/motor neuron disease. (21249659)
2011
13
Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease. (21349440)
2011
14
Motor unit number index (MUNIX): principle, method, and findings in healthy subjects and in patients with motor neuron disease. (20976783)
2010
15
Ubiquitin-positive tau-negative intraneuronal inclusions in dementia with motor neuron disease. (20500451)
2010
16
Semantic dementia combined with motor neuron disease. (19800241)
2009
17
Motor neuron disease features in a patient with neuroborreliosis and a cervical anterior horn lesion. (19670562)
2009
18
Activated protein C therapy slows ALS-like disease in mice by transcriptionally inhibiting SOD1 in motor neurons and microglia cells. (19841542)
2009
19
Severe subcortical TDP-43 pathology in sporadic frontotemporal lobar degeneration with motor neuron disease. (18004574)
2008
20
TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease. (17492294)
2007
21
ALS--motor neuron disease: mechanism and development of new therapies. (18997893)
2007
22
Change in blood antioxidant status of horses moved from a stable following diagnosis of equine motor neuron disease. (18050798)
2007
23
Motor neuron disease in methamphetamine abusers. (17453645)
2007
24
Treatment for spasticity in amyotrophic lateral sclerosis/motor neuron disease. (16437474)
2006
25
Glutamate AMPA receptors change in motor neurons of SOD1G93A transgenic mice and their inhibition by a noncompetitive antagonist ameliorates the progression of amytrophic lateral sclerosis-like disease. (16323214)
2006
26
Ubiquitin-positive inclusions and progression of pathology in frontotemporal dementia and motor neurone disease identifies a group with mainly early pathology. (16409556)
2006
27
Mouse motor neuron disease caused by truncated SOD1 with or without C-terminal modification. (15857664)
2005
28
Mutation of Vps54 causes motor neuron disease and defective spermiogenesis in the wobbler mouse. (16244655)
2005
29
A novel mutation (K317M) in the MAPT gene causes FTDP and motor neuron disease. (15883319)
2005
30
Metabolomic analysis and signatures in motor neuron disease. (18820733)
2005
31
Motor neuron disease group accompanied by inclusions of unidentified protein signaled by ubiquitin. (15139588)
2004
32
The future of motor neuron disease: the challenge is in the genes. (15083302)
2004
33
Relevance of oxidative injury in the pathogenesis of motor neuron diseases. (14753657)
2003
34
Increased expression level of the splicing variant of SIP1 in motor neuron diseases. (11943600)
2002
35
Ubiquitin-only intraneuronal inclusion in the substantia nigra is a characteristic feature of motor neurone disease with dementia. (11972798)
2002
36
Distinctive abnormalities of motor axonal strength-duration properties in multifocal motor neuropathy and in motor neurone disease. (12390974)
2002
37
Compound heterozygosity with two novel mutations in the HEXB gene produces adult Sandhoff disease presenting as a motor neuron disease phenotype. (11897243)
2002
38
Treatment of motor neuron disease with qi-invigorating herbs--a report of 31 cases. (16579087)
2002
39
Immunohistochemical and ultrastructural study of basophilic inclusions in adult-onset motor neuron disease. (11563638)
2001
40
Cognitive impairment in motor neuron disease with bulbar onset. (11465929)
2001
41
Motor neuron disease after electric injury. (11459909)
2001
42
Motor neuron disease. (11345011)
2001
43
Clinicopathological study of atypical motor neuron disease with vertical gaze palsy and ballism. (10965806)
2000
44
Familial frontotemporal dementia with ubiquitin inclusion bodies and without motor neuron disease. (10985702)
2000
45
A novel missense point mutation (S134N) of the Cu/Zn superoxide dismutase gene in a patient with familial motor neuron disease. (8990014)
1997
46
Parathyroid adenoma masquerading as motor neurone disease. (9313992)
1997
47
Localization and expression of ciliary neurotrophic factor (CNTF) in postmortem sciatic nerve from patients with motor neuron disease and diabetic neuropathy. (8759781)
1996
48
Motor neuron disease and model systems: aetiologies, mechanisms and therapies. (8866125)
1996
49
Superoxide dismutase (glu100-->gly) in a family with inherited motor neuron disease: detection of mutant superoxide dismutase activity and the presence of heterodimers. (7624031)
1995
50
Pontocerebellar hypoplasia associated with infantile motor neuron disease (Norman's disease). (2315227)
1990

Genetic Variations for Motor Neuron Disease

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Expression for genes affiliated with Motor Neuron Disease

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1BioGPS, 15Gene Expression Omnibus DataSets
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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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Sources:
37NCBI BioSystems Database, 51QIAGEN, 29KEGG, 4Cell Signaling Technology, 53Reactome, 12EMD Millipore
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Compounds for genes affiliated with Motor Neuron Disease

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Sources:
44Novoseek, 24HMDB, 49PharmGKB, 11DrugBank, 28IUPHAR, 59Tocris Bioscience
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Compounds related to Motor Neuron Disease according to GeneCards/GeneDecks:

(show top 50)    (show all 57)
idCompoundScoreTop Affiliating Genes
16-hydroxydopamine4410.9SOD1, RPS27A, GDNF, SOD2
2superoxide44 2411.9NEFH, ALS2, TARDBP, GDNF, CNTF, SYP
3dicoumarol4410.9SOD1, RPS27A, SOD2
4mntmpyp4410.9SOD2, SOD1
5methamphetamine44 49 1112.9SOD2, RPS27A, CNTF, PVALB
6selegiline44 1111.9SOD2, SOD1, GDNF
7dopamine44 28 11 2413.9CNTF, SOD2, GDNF, PVALB, RPS27A
8acetylcholine44 49 28 11 2414.9SYP, CNTF, SOD2, GDNF, PVALB, RPS27A
9nsc344410.9SOD1, SMN2
10ganglioside4410.9HEXA, HEXB, SYP, CNTF, GDNF
11guanine44 11 2412.9SYP, RPS27A, ALS2, SOD2, RAB5A
12g(m2) ganglioside4410.9HEXA, HEXB
13gaba4410.9CNTF, RPS27A, GDNF, PVALB, SYP
14h2o24410.9SOD1, SYP, SOD2, GDNF, RPS27A, CNTF
15silver4410.9SYP, RPS27A, PVALB, BSCL2
16jw 4805910.9HEXB, HEXA
17tyrosine4410.8NEFH, RAB5A, CNTF, GDNF, PVALB, SMN1
18choline44 11 2412.8CNTF, GDNF, SYP, PVALB
19flurofamide5910.8HEXA, HEXB
20rotenone4410.8RPS27A
21paraffin4410.8RPS27A, NEFH, SYP, PVALB, SOD2
22cysteine4410.8GDNF, NAIP, SYP, SOD1, SOD2, RPS27A
23thiamet g5910.8HEXA, HEXB
24(z)-pugnac5910.8HEXA, HEXB
25zinc superoxide4410.8SOD1, SOD2
26mptp4410.8RPS27A, GDNF, SOD2
27wwl 705910.8HEXB, HEXA
28pyrogallol4410.8SOD2, SOD1
29zinc44 2411.8RAB5A, GDNF, SOD1, SOD2, PVALB, RPS27A
30propyl gallate4410.8SOD2, SOD1
31nitric oxide44 11 2412.8CNTF, SOD2, GDNF, SYP, SOD1, PVALB
32cisplatin44 49 59 1113.8RPS27A, SOD2, SOD1, NAIP, SYP
33glutamate4410.8CNTF, GDNF
34creatinine4410.8GDNF, SYP, SOD1, SOD2, RPS27A
35lipid4410.8SOD1, HEXA, SYP, SOD2, GDNF, CNTF
36guanidine hydrochloride4410.8CNTF, RPS27A, SOD2
37dmpo4410.8SOD1, SOD2
383-nitrotyrosine4410.7SOD2, SOD1
39il 114410.7CNTF, CTF1
40aminoglycosides4410.7SMN1, GDNF
41n acetylcysteine4410.7RPS27A, SOD1, CNTF, SOD2
42aclarubicin4410.7SMN2, SMN1
43glutathion4410.7SOD2, SOD1
444-hydroxynonenal44 2411.6SOD2, SOD1, RPS27A
45butyrate4410.6SOD2, SMN1, RPS27A, SMN2
46riluzole44 28 1112.5SOD1, GDNF
473-amino-1,2,4-triazole4410.5SOD2, SOD1
48nmda44 2811.4RPS27A, SYP, PVALB
49melittin4410.4PVALB, RPS27A
50levodopa44 1111.2GDNF, SOD1, RPS27A

GO Terms for genes affiliated with Motor Neuron Disease

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16Gene Ontology
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Cellular components related to Motor Neuron Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1neurofilamentGO:00588310.5INA, NEFH

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

(show all 17)
idNameGO IDScoreTop Affiliating Genes
1negative regulation of neuron apoptotic processGO:04352410.8NAIP, GDNF, CNTF, SOD1, SOD2
2neurofilament cytoskeleton organizationGO:06005210.7SOD1, NEFH, INA
3locomotory behaviorGO:00762610.7ALS2, SOD1, SOD2, HEXB
4cell deathGO:00821910.7ALS2, HEXA, HEXB, BSCL2, NEFH, DCTN1
5nervous system developmentGO:00739910.7CTF1, NEFH, DCTN1, GDNF, NAIP, INA
6neuron developmentGO:04866610.7CNTF, CTF1, SOD2
7lipid storageGO:01991510.7HEXA, HEXB, BSCL2
8hydrogen peroxide biosynthetic processGO:05066510.7SOD2, SOD1
9ganglioside catabolic processGO:00668910.7HEXB, HEXA
10removal of superoxide radicalsGO:01943010.7SOD1, SOD2
11keratan sulfate catabolic processGO:04234010.6HEXB, HEXA
12hyaluronan catabolic processGO:03021410.6HEXA, HEXB
13chondroitin sulfate catabolic processGO:03020710.5HEXA, HEXB
14superoxide anion generationGO:04255410.5SOD1, SOD2
15sensory perception of soundGO:00760510.5SOD1, HEXB, HEXA
16regulation of mitochondrial membrane potentialGO:05188110.4SOD2, SOD1
17superoxide metabolic processGO:00680110.2SOD1, SOD2

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

idNameGO IDScoreTop Affiliating Genes
1identical protein bindingGO:04280210.5SMN2, TARDBP, SYP, SOD1, SOD2
2beta-N-acetylhexosaminidase activityGO:00456310.4HEXB, HEXA
3superoxide dismutase activityGO:00478410.2SOD1, SOD2

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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet