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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Sources:
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
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

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

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
Trail Making Test error analysis in classic motor neuron disease. (23161259)
2013
2
Mitochondrial genome aberrations in skeletal muscle of patients with motor neuron disease. (23134511)
2013
3
Repetitive transcranial magnetic stimulation for the treatment of amyotrophic lateral sclerosis or motor neuron disease. (23728676)
2013
4
Homozygous survival motor neuron 2 gene deletion and sporadic lower motor neuron disease in children: case report and literature review. (22628217)
2013
5
Management of sialorrhoea in motor neuron disease: A survey of current UK practice. (23647474)
2013
6
Longitudinal tracking of human fetal cells labeled with super paramagnetic iron oxide nanoparticles in the brain of mice with motor neuron disease. (22384217)
2012
7
Ablation of proliferating cells in the CNS exacerbates motor neuron disease caused by mutant superoxide dismutase. (22523565)
2012
8
Frontotemporal dementia and motor neuron disease: report of 3 cases in Taiwan and literature review. (22009125)
2011
9
Do patients with motor neuron disease suffer from disorders of taste or smell? (21554187)
2011
10
Familial amyotrophic lateral sclerosis with a novel G85S mutation of superoxide dismutase 1 gene: clinical features of lower motor neuron disease. (20075587)
2010
11
Madras motor neuron disease in Turkey. (19922122)
2009
12
Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease. (18425913)
2008
13
Genetically confirmed Huntington's disease masquerading as motor neuron disease. (18186118)
2008
14
Drug therapy for pain in amyotrophic lateral sclerosis or motor neuron disease. (18646122)
2008
15
Disruption of neurofilament network with aggregation of light neurofilament protein: a common pathway leading to motor neuron degeneration due to Charcot-Marie-Tooth disease-linked mutations in NFL and HSPB1. (17881652)
2007
16
Motor neuron disease recovery associated with IV ceftriaxone and anti-Babesia therapy. (17212618)
2007
17
Pick's disease with Pick bodies: an unusual autopsy case showing degeneration of the pontine nucleus, dentate nucleus, Clarke's column, and lower motor neuron. (17319287)
2007
18
Absence of paraneoplastic antineuronal antibodies in sera of 145 patients with motor neuron disease. (17314193)
2007
19
Antioxidant treatment for amyotrophic lateral sclerosis / motor neuron disease. (17253482)
2007
20
An autopsy case of frontotemporal dementia with severe dysarthria and motor neuron disease showing numerous basophilic inclusions. (17080724)
2006
21
Hypoglossal neuropathy in Lewis-Sumner syndrome masquerading as motor neuron disease. (16832106)
2006
22
A gene for an autosomal recessive lower motor neuron disease with childhood onset maps to 1p36. (16728649)
2006
23
Motor neuron disease in a patient with a mitochondrial tRNAIle mutation. (16358336)
2006
24
Senataxin, the yeast Sen1p orthologue: characterization of a unique protein in which recessive mutations cause ataxia and dominant mutations cause motor neuron disease. (16644229)
2006
25
A retrospective analysis of sleep quality and survival with domiciliary ventilatory support in motor neuron disease. (16753974)
2006
26
Inherited motor neuron disease in domestic cats: a model of spinal muscular atrophy. (15635053)
2005
27
Analysis of heavy neurofilament subunit gene polymorphism in Russian patients with sporadic motor neuron disease (MND). (14722583)
2004
28
Frontotemporal lobar degeneration with motor neuron disease-type inclusions predominates in 76 cases of frontotemporal degeneration. (15351890)
2004
29
Axonal ionic pathophysiology in human peripheral neuropathy and motor neuron disease. (16181085)
2004
30
Rapidly progressive aphasic dementia with motor neuron disease: a distinctive clinical entity. (14560061)
2004
31
Alpha-synuclein in motor neuron disease: an immunohistologic study. (14648076)
2004
32
PQBP-1 transgenic mice show a late-onset motor neuron disease-like phenotype. (12651867)
2003
33
Morphometrical reappraisal of motor neuron system of Pick's disease and amyotrophic lateral sclerosis with dementia. (12070660)
2002
34
Motor neuron disease presenting with slow saccades and dementia. (12495578)
2002
35
Models of care for motor neuron disease: setting standards. (12710506)
2002
36
Adult Sandhoff disease presented as a motor neuron disease phenotype with slow progression]. (11433765)
2001
37
Surface electromyography using electrode arrays: a study of motor neuron disease. (11180205)
2001
38
Clinical implications of the genetics of ALS and other motor neuron diseases. (11460829)
2001
39
Striatonigral degeneration with motor neuron disease. (10896276)
2000
40
Motor neuron disease: classification and nomenclature. (11467047)
2000
41
Congenital cytoplasmic body myopathy with survival motor neuron gene deletion or Werdnig-Hoffmann disease. (9748045)
1998
42
Coadministration of interleukin-6 (IL-6) and soluble IL-6 receptor delays progression of wobbler mouse motor neuron disease. (8836549)
1996
43
Malignant hyperthermia susceptibility in a patient with concomitant motor neuron disease. (8902717)
1996
44
Motor neuron disease with neurofibrillary tangles in a non-Guamanian patient. (7572072)
1995
45
The effects of ciliary neurotrophic factor on motor dysfunction in wobbler mouse motor neuron disease. (8053649)
1994
46
Preferential beta-hexosaminidase (Hex) A (alpha beta) formation in the absence of beta-Hex B (beta beta) due to heterozygous point mutations present in beta-Hex beta-chain alleles of a motor neuron disease patient. (8106452)
1994
47
Parvalbumin and calbindin D-28k in the human motor system and in motor neuron disease. (8232749)
1993
48
Kennedy's disease: genetic diagnosis of an inherited form of motor neuron disease. (8517843)
1993
49
Progressive bulbar palsy: a case report of a type of motor neuron disease presenting with oral symptoms. (2304745)
1990
50
Neurophysiological study in chronic GM2 gangliosidosis (hexosaminidase A and B deficiency), with motor neuron disease phenotype. (2793417)
1989

Genetic Variations for Motor Neuron Disease

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Expression for genes affiliated with Motor Neuron Disease

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Sources:
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-hydroxydopamine4410.9RPS27A, SOD2, SOD1, GDNF
2superoxide44 2411.9ALS2, SOD2, SOD1, NEFH, SYP, CNTF
3dicoumarol4410.9RPS27A, SOD2, SOD1
4mntmpyp4410.9SOD2, SOD1
5methamphetamine44 49 1112.9CNTF, SOD2, PVALB, RPS27A
6selegiline44 1111.9GDNF, SOD1, SOD2
7dopamine44 28 11 2413.9RPS27A, PVALB, SOD2, CNTF, GDNF
8acetylcholine44 49 28 11 2414.9RPS27A, PVALB, SOD2, SYP, CNTF, GDNF
9nsc344410.9SOD1, SMN2
10ganglioside4410.9HEXA, HEXB, SYP, CNTF, GDNF
11guanine44 11 2412.9ALS2, RAB5A, SYP, SOD2, RPS27A
12g(m2) ganglioside4410.9HEXA, HEXB
13gaba4410.9RPS27A, PVALB, SYP, CNTF, GDNF
14h2o24410.9RPS27A, SOD2, SOD1, SYP, CNTF, GDNF
15silver4410.9SYP, BSCL2, PVALB, RPS27A
16jw 4805910.9HEXA, HEXB
17tyrosine4410.8RPS27A, PVALB, SOD2, NEFH, SYP, CNTF
18choline44 11 2412.8PVALB, SYP, CNTF, GDNF
19flurofamide5910.8HEXA, HEXB
20rotenone4410.8RPS27A
21paraffin4410.8RPS27A, PVALB, SOD2, NEFH, SYP
22cysteine4410.8RPS27A, SOD2, SOD1, SYP, GDNF, NAIP
23thiamet g5910.8HEXA, HEXB
24(z)-pugnac5910.8HEXA, HEXB
25pyrogallol4410.8SOD2, SOD1
26mptp4410.8GDNF, SOD2, RPS27A
27wwl 705910.8HEXA, HEXB
28zinc superoxide4410.8SOD2, SOD1
29zinc44 2411.8RPS27A, PVALB, SOD2, SOD1, GDNF, RAB5A
30propyl gallate4410.8SOD1, SOD2
31nitric oxide44 11 2412.8PVALB, SOD2, SOD1, SYP, CNTF, GDNF
32cisplatin44 49 59 1113.8RPS27A, SOD2, SOD1, SYP, NAIP
33glutamate4410.8GDNF, CNTF
34creatinine4410.8RPS27A, SOD2, SOD1, SYP, GDNF
35lipid4410.8HEXA, SOD2, SOD1, SYP, CNTF, GDNF
36guanidine hydrochloride4410.8CNTF, SOD2, RPS27A
37dmpo4410.8SOD2, SOD1
38il 114410.7CTF1, CNTF
393-nitrotyrosine4410.7SOD2, SOD1
40aminoglycosides4410.7GDNF, SMN1
41n acetylcysteine4410.7CNTF, SOD1, SOD2, RPS27A
42aclarubicin4410.7SMN1, SMN2
43glutathion4410.7SOD2, SOD1
444-hydroxynonenal44 2411.6SOD1, SOD2, RPS27A
45butyrate4410.6RPS27A, SOD2, SMN1, SMN2
46riluzole44 28 1112.5SOD1, GDNF
47melittin4410.5RPS27A, PVALB
48nmda44 2811.4SYP, PVALB, RPS27A
493-amino-1,2,4-triazole4410.4SOD2, SOD1
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