MCID: MTR014
MIFTS: 56

Motor Neuron Disease malady

Neuronal category

Summaries for Motor Neuron Disease

Sources:
8Disease Ontology, 44NINDS, 64Wikipedia, 33MalaCards
See all sources

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NINDS:44 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, and related mouse phenotypes are nervous system and behavior/neurological.

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

Wikipedia:64 The motor neuron diseases (MND) are a group of neurological disorders that selectively affect motor... more...

Aliases & Classifications for Motor Neuron Disease

Sources:
8Disease Ontology, 44NINDS, 10DISEASES, 45Novoseek, 31LifeMap Discovery™, 61UMLS, 27ICD9CM, 57SNOMED-CT, 35MeSH, 25ICD10
See all sources

Classifications:

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


Aliases & Descriptions:

motor neuron disease 8 10 45 31 61
motor neuron diseases 44


External Ids:

Disease Ontology8 DOID:231
ICD9CM27 335.2
MeSH35 D016472
ICD1025 G12.2

Related Diseases for Motor Neuron Disease

Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 150)
idRelated DiseaseScoreTop Affiliating Genes
1amyotrophic lateral sclerosis31.5DCTN1, NEFH
2lateral sclerosis31.5ALS2, SOD1
3frontotemporal dementia31.2SOD1, RPS27A, INA, TARDBP
4parkinson's disease30.8RPS27A, GDNF
5muscular atrophy30.7SMN2, SMN1, NAIP
6spinal muscular atrophy30.6SMN1, SMN2
7sandhoff disease30.5HEXA, HEXB
8progressive supranuclear palsy30.4TARDBP
9semantic dementia30.3RPS27A
10adenoma30.3GDNF, SYP
11pick's disease30.1RPS27A, TARDBP
12alzheimer's disease30.1GDNF, INA, PVALB, SOD2, SOD1, NEFH
13n syndrome10.7
14madras motor neuron disease10.7
15spinal cord disease10.7
16brain disease10.6
17ataxia10.4
18spasticity10.4
19progressive muscular atrophy10.4
20anterior horn cell disease10.4
21chorea10.4
22axonal neuropathy10.3
23mu chain disease10.3
24spinocerebellar degeneration10.3
25tauopathy10.3
26monomelic amyotrophy10.3
27multifocal motor neuropathy10.3
28m�ni�re disease10.3
29benign focal amyotrophy10.3
30werdnig-hoffmann disease10.2
31striatonigral degeneration10.2
32spondylosis10.2
33monoclonal paraproteinemia10.2
34kennedy's disease10.2
35motor peripheral neuropathy10.2
36progressive bulbar palsy10.2
37apraxia10.2
38autosomal recessive disease10.2
39spinocerebellar ataxia10.2
40acanthocytosis10.2
41brown syndrome10.2
42fazio londe syndrome10.2
43frontotemporal dementia, ubiquitin-positive10.2
44lathyrism10.2
45amyotrophic lateral sclerosis with frontotemporal dementia10.2
46guillain-barr� syndrome10.2
47werdnig-hoffman disease10.2
48amyotrophic lateral sclerosis 2010.2
49myokymia10.2
50nutrition disease10.2

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



Diseases related to motor neuron disease

Clinical Features for Motor Neuron Disease

Drugs & Therapeutics for Motor Neuron Disease

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 31LifeMap Discovery™, 61UMLS, 41NDF-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

Anatomical Context for Motor Neuron Disease

Sources:
31LifeMap Discovery™, 14FMA
See all sources

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

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

Publications for Motor Neuron Disease

Sources:
51PubMed
See all sources

Articles related to Motor Neuron Disease:

(show top 50)    (show all 660)
idTitleAuthorsYear
1
Distinctive features of the D101N and D101G variants of superoxide dismutase 1; two mutations that produce rapidly progressing motor neuron disease. (24032979)
2014
2
Orofacial apraxia in motor neuron disease. (23569452)
2013
3
Treatment of respiratory impairment in patients with motor neuron disease in the Netherlands: patient preference and timing of referral. (23398243)
2013
4
Co-Transplantation of GDNF-Overexpressing Neural Stem Cells and Fetal Dopaminergic Neurons Mitigates Motor Symptoms in a Rat Model of Parkinson's Disease. (24312503)
2013
5
The role of facemask spirometry in motor neuron disease. (22843559)
2013
6
The initiation of non-invasive ventilation for patients with motor neuron disease: patient and carer perceptions of obstacles and outcomes. (22953736)
2013
7
Motor neuron disease and frontotemporal dementia: sometimes related, sometimes not. (24246281)
2013
8
The impact of perceived lack of support provided by health and social care services to caregivers of people with motor neuron disease. (22292844)
2012
9
Association of oxidative stress with motor neuron disease in horses. (23176423)
2012
10
A systematic review of behavioural changes in motor neuron disease. (22424127)
2012
11
Changing mortality for motor neuron disease in France (1968-2007): an age-period-cohort analysis. (21674216)
2011
12
Motor neuron disease: systematic reviews of treatment for ALS and SMA. (20015852)
2010
13
Posteriori conformal radiotherapy using three-dimensional dosimetric reconstitution in a survivor of adult-onset Hodgkin's disease for definitive diagnosis of lower motor neuron disease. (20713856)
2010
14
An evaluation of neurophysiological criteria used in the diagnosis of motor neuron disease. (20522872)
2010
15
Motor neuron disease and frontotemporal dementia: One, two, or three diseases? (21369423)
2010
16
Public awareness of motor neuron disease. (20672874)
2010
17
Survival profiles of patients with frontotemporal dementia and motor neuron disease. (19901167)
2009
18
Motor neuron disease: the bare essentials. (19762894)
2009
19
Motor-neuron disease: Rogue gene in the family. (19325616)
2009
20
Motor unit number estimation in the assessment of performance and function in motor neuron disease. (18625413)
2008
21
Forme fruste or incipient form of widespread-type amyotrophic lateral sclerosis, or motor neuron disease with pallido-nigro-luysian atrophy? An autopsy case report. (18179405)
2008
22
Role of complement in motor neuron disease: animal models and therapeutic potential of complement inhibitors. (19025120)
2008
23
Nogo-A marks motor neuron disease. (17474106)
2007
24
The complex relation between genotype and phenotype in motor neuron disease. (17469207)
2007
25
Evaluation of the risk of motor neuron disease in horses fed a diet low in vitamin E and high in copper and iron. (16426221)
2006
26
Nocturnal hypoxia in motor neuron disease is not predicted by standard respiratory function tests. (16780447)
2006
27
Restoration of aspartoacylase activity in CNS neurons does not ameliorate motor deficits and demyelination in a model of Canavan disease. (15851013)
2005
28
Primary hyperparathyroidism simulating motor neuron disease: case report. (15830084)
2005
29
Treatment for spasticity in amyotrophic lateral sclerosis/motor neuron disease. (14974059)
2004
30
Recessive motor neuron diseases: mutations in the ALS2 gene and molecular pathogenesis for the upper motor neurodegeneration]. (15651293)
2004
31
Adult onset Krabbe disease may mimic motor neurone disease. (12948479)
2003
32
Pathoetiology of motor neuron disease: new insights from genetics and animal models. (12763330)
2003
33
The relationship between extramotor ubiquitin-immunoreactive neuronal inclusions and dementia in motor neuron disease. (12536219)
2003
34
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
35
Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading. (11889469)
2002
36
Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). (12076411)
2002
37
Recombinant human insulin-like growth factor I (rhIGF-I) for amyotrophic lateral sclerosis/motor neuron disease. (12137643)
2002
38
Missense mutation in the tubulin-specific chaperone E (Tbce) gene in the mouse mutant progressive motor neuronopathy, a model of human motoneuron disease. (12446740)
2002
39
Chorea in motor neuron disease. (12465097)
2002
40
Effects of cardiotrophin-1 (CT-1) in a mouse motor neuron disease. (11360260)
2001
41
Motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration. (10435507)
1999
42
Detection of the survival motor neuron (SMN) genes by FISH: further evidence for a role for SMN2 in the modulation of disease severity in SMA patients. (10556301)
1999
43
Gallyas- and tau-positive glial structures in motor neuron disease with dementia. (10505430)
1999
44
Amyloid neuropathy simulating lower motor neuron disease. (9710046)
1998
45
Characterisation of the distribution of choline acetyltransferase messenger RNA in human spinal cord and its depletion in motor neurone disease. (1469422)
1992
46
Ubiquitin in motor neuron disease: study at the light and electron microscope. (1648124)
1991
47
Separating motor neuron diseases from pure motor neuropathies. Multifocal motor neuropathy with persistent conduction block. (1853773)
1991
48
Nerve conduction studies in upper limbs of patients with cervical spondylosis and motor neurone disease. (3176881)
1988
49
The effect of peripheral nerve injury on immature motor and sensory neurons and on muscle fibres. Possible relation to the histogenesis of Werdnig-Hoffmann disease. (3231961)
1988
50
Abnormalities of lipoprotein and carbohydrate metabolism in degenerative diseases of the nervous system--motor neuron disease and spinocerebellar degeneration. (6346585)
1983

Genetic Variations for Motor Neuron Disease

Expression for genes affiliated with Motor Neuron Disease

Sources:
1BioGPS, 15Gene 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

Sources:
38NCBI BioSystems Database, 52QIAGEN, 30KEGG, 4Cell Signaling Technology, 54Reactome, 12EMD Millipore
See all sources

Compounds for genes affiliated with Motor Neuron Disease

Sources:
45Novoseek, 24HMDB, 50PharmGKB, 11DrugBank, 29IUPHAR, 60Tocris Bioscience
See all sources

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

(show top 50)    (show all 57)
idCompoundScoreTop Affiliating Genes
16-hydroxydopamine4510.9SOD1, RPS27A, GDNF, SOD2
2superoxide45 2411.9NEFH, ALS2, TARDBP, GDNF, CNTF, SYP
3dicoumarol4510.9SOD1, RPS27A, SOD2
4mntmpyp4510.9SOD2, SOD1
5methamphetamine45 50 1112.9SOD2, RPS27A, CNTF, PVALB
6selegiline45 1111.9SOD2, SOD1, GDNF
7dopamine45 29 11 2413.9CNTF, SOD2, GDNF, PVALB, RPS27A
8acetylcholine45 50 29 11 2414.9SYP, CNTF, SOD2, GDNF, PVALB, RPS27A
9nsc344510.9SOD1, SMN2
10ganglioside4510.9HEXA, HEXB, SYP, CNTF, GDNF
11guanine45 11 2412.9SYP, RPS27A, ALS2, SOD2, RAB5A
12g(m2) ganglioside4510.9HEXA, HEXB
13gaba4510.9CNTF, RPS27A, GDNF, PVALB, SYP
14h2o24510.9SOD1, SYP, SOD2, GDNF, RPS27A, CNTF
15silver4510.9SYP, RPS27A, PVALB, BSCL2
16jw 4806010.9HEXB, HEXA
17tyrosine4510.8NEFH, RAB5A, CNTF, GDNF, PVALB, SMN1
18choline45 11 2412.8CNTF, GDNF, SYP, PVALB
19flurofamide6010.8HEXA, HEXB
20rotenone4510.8RPS27A
21paraffin4510.8RPS27A, NEFH, SYP, PVALB, SOD2
22cysteine4510.8GDNF, NAIP, SYP, SOD1, SOD2, RPS27A
23thiamet g6010.8HEXA, HEXB
24(z)-pugnac6010.8HEXA, HEXB
25zinc superoxide4510.8SOD1, SOD2
26mptp4510.8RPS27A, GDNF, SOD2
27wwl 706010.8HEXB, HEXA
28pyrogallol4510.8SOD2, SOD1
29zinc45 2411.8RAB5A, GDNF, SOD1, SOD2, PVALB, RPS27A
30propyl gallate4510.8SOD2, SOD1
31nitric oxide45 11 2412.8CNTF, SOD2, GDNF, SYP, SOD1, PVALB
32cisplatin45 50 60 1113.8RPS27A, SOD2, SOD1, NAIP, SYP
33glutamate4510.8CNTF, GDNF
34creatinine4510.8GDNF, SYP, SOD1, SOD2, RPS27A
35lipid4510.8SOD1, HEXA, SYP, SOD2, GDNF, CNTF
36guanidine hydrochloride4510.8CNTF, RPS27A, SOD2
37dmpo4510.8SOD1, SOD2
383-nitrotyrosine4510.7SOD2, SOD1
39il 114510.7CNTF, CTF1
40aminoglycosides4510.7SMN1, GDNF
41n acetylcysteine4510.7RPS27A, SOD1, CNTF, SOD2
42aclarubicin4510.7SMN2, SMN1
43glutathion4510.7SOD2, SOD1
444-hydroxynonenal45 2411.6SOD2, SOD1, RPS27A
45butyrate4510.6SOD2, SMN1, RPS27A, SMN2
46riluzole45 29 1112.5SOD1, GDNF
473-amino-1,2,4-triazole4510.5SOD2, SOD1
48nmda45 2911.4RPS27A, SYP, PVALB
49melittin4510.4PVALB, RPS27A
50levodopa45 1111.2GDNF, SOD1, RPS27A

GO Terms for genes affiliated with Motor Neuron Disease

Sources:
16Gene Ontology
See all sources

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:04352410.8SOD1, CNTF, GDNF, NAIP, SOD2
2neurofilament cytoskeleton organizationGO:06005210.7SOD1, NEFH, INA
3locomotory behaviorGO:00762610.7HEXB, ALS2, SOD1, SOD2
4cell deathGO:00821910.7ALS2, SMN2, TARDBP, DCTN1, NEFH, BSCL2
5nervous system developmentGO:00739910.7CTF1, NEFH, DCTN1, GDNF, INA, RAB5A
6neuron developmentGO:04866610.7CTF1, CNTF, SOD2
7lipid storageGO:01991510.7BSCL2, HEXB, HEXA
8hydrogen peroxide biosynthetic processGO:05066510.7SOD1, SOD2
9ganglioside catabolic processGO:00668910.7HEXA, HEXB
10removal of superoxide radicalsGO:01943010.7SOD2, SOD1
11keratan sulfate catabolic processGO:04234010.6HEXB, HEXA
12hyaluronan catabolic processGO:03021410.6HEXB, HEXA
13chondroitin sulfate catabolic processGO:03020710.5HEXB, HEXA
14superoxide anion generationGO:04255410.5SOD1, SOD2
15sensory perception of soundGO:00760510.5SOD1, HEXB, HEXA
16regulation of mitochondrial membrane potentialGO:05188110.4SOD1, SOD2
17superoxide metabolic processGO:00680110.2SOD2, SOD1

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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  • Antibodies

Sources for Motor Neuron Disease

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet