PLS
MCID: LTR001
MIFTS: 52

Lateral Sclerosis (PLS) malady

Neuronal category

Summaries for Lateral Sclerosis

Sources:
43NIH Rare Diseases, 44NINDS, 64Wikipedia, 47OMIM, 33MalaCards
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NIH Rare Diseases:43 Primary lateral sclerosis is a type of motor neuron disease, where nerve cells that control voluntary muscle movement breakdown and die. in primary lateral sclerosis only the upper motor neurons in the brain are affected. symptoms often begin with problems in the legs (e.g., weakness, stiffness, spasticity, and balance problems), but may also start with hand clumsiness and changes in speech. the symptoms worsen gradually over time, however people with this condition have a normal life expectancy. progression of symptoms varies from person to person, some people retain the ability to walk without assistance, others eventually require assistive devices such as canes or wheelchairs. diagnosis requires extensive testing to exclude other diseases. treatment may include baclofen and tizanidine to reduce spasticity, quinine or phenytoin to reduce cramps, as well as physical and speech therapy as required. last updated: 1/22/2010

MalaCards: Lateral Sclerosis, also known as primary lateral sclerosis, is related to amyotrophic lateral sclerosis and frontotemporal dementia. An important gene associated with Lateral Sclerosis is FIG4 (FIG4 homolog, SAC1 lipid phosphatase domain containing (S. cerevisiae)), and among its related pathways is Pathogenesis of ALS. Affiliated tissues include skin, liver and tongue.

NINDS:44 Primary lateral sclerosis (PLS) is a rare neuromuscular disease with slowly progressive weakness in voluntary muscle movement. PLS belongs to a group of disorders known as motor neuron diseases. PLS affects the upper motor neurons (also called corticospinal neurons) in the arms, legs, and face.

Wikipedia:64 Amyotrophic lateral sclerosis (ALS) – also referred to as motor neurone disease (MND) in most... more...

Description from OMIM:47 611637

Aliases & Classifications for Lateral Sclerosis

Sources:
8Disease Ontology, 10DISEASES, 61UMLS, 43NIH Rare Diseases, 44NINDS, 45Novoseek, 49Orphanet, 47OMIM, 27ICD9CM, 57SNOMED-CT, 35MeSH, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
primary lateral sclerosis:
Inheritance: Autosomal recessive,Sporadic; Prevalence: 1-9/100000; Age of onset: Variable; Age of death: Normal


Aliases & Descriptions:

lateral sclerosis 8 10 61
primary lateral sclerosis 8 43 44 45 49 47
adult-onset primary lateral sclerosis 49
primary lateral sclerosis, adult, 1 61
papillon-lefevre disease 61
adult-onset pls 49
pls 49


External Ids:

Disease Ontology8 DOID:230
ICD9CM27 335.24
MeSH35 D016472
SNOMED-CT57 81211007
OMIM47 611637
ICD10 via Orphanet26 G12.2
SNOMED-CT via Orphanet58 81211007

Related Diseases for Lateral Sclerosis

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the juvenile primary lateral sclerosis family:

lateral sclerosis primary lateral sclerosis, adult, 1

Diseases related to Lateral Sclerosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 185)
idRelated DiseaseScoreTop Affiliating Genes
1amyotrophic lateral sclerosis32.4NEFH, PRPH
2frontotemporal dementia31.2FUS, C9orf72, SOD1
3juvenile primary lateral sclerosis10.7
4papillon-lefevre disease10.7
5amyotrophic lateral sclerosis-parkinsonism/dementia complex10.6
6muscular atrophy10.6
7parkinson's disease10.6
8amyotrophic lateral sclerosis type 210.5
9amyotrophic lateral sclerosis, type 410.5
10alzheimer's disease10.5
11amyotrophic lateral sclerosis 2010.5
12amyotrophic lateral sclerosis type 810.5
13spinal muscular atrophy10.5
14progressive muscular atrophy10.4
15amyotrophic lateral sclerosis type 1110.4
16amyotrophic lateral sclerosis type 910.4
17amyotrophic lateral sclerosis 1910.4
18amyotrophic lateral sclerosis type 310.4
19amyotrophic lateral sclerosis type 710.4
20werdnig-hoffmann disease10.4
21progressive supranuclear palsy10.4
22amyotrophic lateral sclerosis type 1010.4
23amyotrophic lateral sclerosis with frontotemporal dementia10.4
24sod1-related amyotrophic lateral sclerosis10.4
25werdnig-hoffman disease10.4
26hereditary spastic paraplegia10.3
27pick's disease10.3
28amyotrophic lateral sclerosis type 510.3
29amyotrophic lateral sclerosis-parkinsonism/dementia complex 110.3
30n syndrome10.3
31amyotrophic lateral sclerosis type 610.3
32multifocal motor neuropathy10.3
33dysphagia10.3
34amyotrophic lateral sclerosis 2, juvenile10.3
35clear cell acanthoma10.3
36pleomorphic liposarcoma10.3
37huntington's disease10.2
38spinal cord disease10.2
39tauopathy10.2
40tardbp-related amyotrophic lateral sclerosis10.2
41spastic paraparesis10.2
42vapb-related amyotrophic lateral sclerosis10.2
43hypoxia10.2
44spasticity10.2
45amyotrophic lateral sclerosis 1210.2
46anosognosia10.2
47anterior horn cell disease10.2
48aphasia10.2
49autosomal recessive juvenile amyotrophic lateral sclerosis10.2
50setx-related amyotrophic lateral sclerosis10.2

Graphical network of the top 20 diseases related to Lateral Sclerosis:



Diseases related to lateral sclerosis

Clinical Features for Lateral Sclerosis

Sources:
47OMIM
See all sources

Clinical features from OMIM:

611637

Clinical synopsis from OMIM:

611637

Drugs & Therapeutics for Lateral Sclerosis

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Lateral Sclerosis

Drug clinical trials:

Search ClinicalTrials for Lateral Sclerosis

Search NIH Clinical Center for Lateral Sclerosis

Search CenterWatch for Lateral Sclerosis

Genetic Tests for Lateral Sclerosis

Anatomical Context for Lateral Sclerosis

Sources:
33MalaCards
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MalaCards organs/tissues related to Lateral Sclerosis:

33
Skin, Liver, Tongue, Spinal cord, Brain, Bone marrow, Whole blood, Cortex, Cerebellum, Smooth muscle, Skeletal muscle, Small intestine, Salivary gland, Breast, Prostate, Monocytes, T cells, B cells, Endothelial, Prefrontal cortex, Temporal lobe, Ciliary ganglion, Amygdala, Dorsal root ganglion, Pituitary

Animal Models for Lateral Sclerosis or affiliated genes

Sources:
28inGenious Targeting Laboratory
See all sources

Publications for Lateral Sclerosis

Sources:
51PubMed
See all sources

Articles related to Lateral Sclerosis:

(show top 50)    (show all 1875)
idTitleAuthorsYear
1
Distributed corpus callosum involvement in amyotrophic lateral sclerosis: a deterministic tractography study using q-ball imaging. (24126610)
2014
2
Advances in Cellular Models to Explore the Pathophysiology of Amyotrophic Lateral Sclerosis. (24198229)
2013
3
Underlying cause and place of death among patients with amyotrophic lateral sclerosis in Taiwan: a population-based study, 2003-2008. (23933623)
2013
4
Macroglossia in amyotrophic lateral sclerosis. (24042440)
2013
5
Health-related quality of life in patients with amyotrophic lateral sclerosis. (23601755)
2013
6
Systemic treatment with adipose-derived mesenchymal stem cells ameliorates clinical and pathological features in the amyotrophic lateral sclerosis murine model. (23727509)
2013
7
Utility of transcranial magnetic stimulation in delineating amyotrophic lateral sclerosis pathophysiology. (24112924)
2013
8
Spinal cord atrophy correlates with disease duration and severity in amyotrophic lateral sclerosis. (24219347)
2013
9
Human marrow stromal cells reduce microglial activation to protect motor neurons in a transgenic mouse model of amyotrophic lateral sclerosis. (23631660)
2013
10
Atypical progressive supranuclear palsy presenting as primary lateral sclerosis. (23570981)
2013
11
Comment on "Anaesthetic management of a patient with amyotrophic lateral sclerosis for transurethral resection of bladder tumour". (24403640)
2013
12
Electrodiagnosis in persons with amyotrophic lateral sclerosis. (23523708)
2013
13
A man with amyotrophic lateral sclerosis uses a mouth pressure microswitch to operate a text messaging system with a word prediction function. (24020877)
2013
14
Surgical improvement of speech disorder caused by amyotrophic lateral sclerosis. (23171742)
2012
15
Ataxin-1 and ataxin-2 intermediate-length PolyQ expansions in amyotrophic lateral sclerosis. (23197749)
2012
16
Founder effect hypothesis of D11Y SOD1 mutation in Italian amyotrophic lateral sclerosis patients. (22292847)
2012
17
Misfolded superoxide dismutase-1 in CSF from amyotrophic lateral sclerosis patients. (21226712)
2011
18
FUS mutations in sporadic amyotrophic lateral sclerosis. (20138404)
2011
19
Novel optineurin mutations in patients with familial and sporadic amyotrophic lateral sclerosis. (21613650)
2011
20
Proteins that bind to misfolded mutant superoxide dismutase-1 in spinal cords from transgenic amyotrophic lateral sclerosis (ALS) model mice. (21493711)
2011
21
Appearance of phagocytic microglia adjacent to motoneurons in spinal cord tissue from a presymptomatic transgenic rat model of amyotrophic lateral sclerosis. (20648658)
2010
22
Inclusion body myositis masquerading as amyotrophic lateral sclerosis. (21059520)
2010
23
Oxidative stress in skeletal muscle stimulates early expression of Rad in a mouse model of amyotrophic lateral sclerosis. (20079427)
2010
24
TDP-43 physically interacts with amyotrophic lateral sclerosis-linked mutant CuZn superoxide dismutase. (20933032)
2010
25
Modification of superoxide dismutase 1 (SOD1) properties by a GFP tag--implications for research into amyotrophic lateral sclerosis (ALS). (20221404)
2010
26
Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules. (20699327)
2010
27
Meta-analysis of vascular endothelial growth factor variations in amyotrophic lateral sclerosis: increased susceptibility in male carriers of the -2578AA genotype. (18413368)
2009
28
Amyotrophic lateral sclerosis linked to a novel SOD1 mutation with muscle mitochondrial dysfunction. (19000626)
2009
29
Activation of signal transducer and activator of transcription-3 in the spinal cord of sporadic amyotrophic lateral sclerosis patients. (19372705)
2009
30
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. (18309045)
2008
31
Development of a high-throughput microarray-based resequencing system for neurological disorders and its application to molecular genetics of amyotrophic lateral sclerosis. (18852346)
2008
32
50bp deletion in the promoter for superoxide dismutase 1 (SOD1) reduces SOD1 expression in vitro and may correlate with increased age of onset of sporadic amyotrophic lateral sclerosis. (18608091)
2008
33
Targeting angiogenin in therapy of amyotropic lateral sclerosis. (18781822)
2008
34
A familial form of pallidoluysionigral degeneration and amyotrophic lateral sclerosis with divergent clinical presentations. (17620990)
2007
35
Elevation of MCP-1 and MCP-1/VEGF ratio in cerebrospinal fluid of amyotrophic lateral sclerosis patients. (17672928)
2007
36
HFE H63D polymorphism is increased in patients with amyotrophic lateral sclerosis of Italian origin. (17308297)
2007
37
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
38
Transferrin localizes in Bunina bodies in amyotrophic lateral sclerosis. (16896902)
2006
39
O-glycosylation of the tail domain of neurofilament protein M in human neurons and in spinal cord tissue of a rat model of amyotrophic lateral sclerosis (ALS). (16006557)
2005
40
Activation of a glycine transporter on spinal cord neurons causes enhanced glutamate release in a mouse model of amyotrophic lateral sclerosis. (12684256)
2003
41
Impairment of mitochondrial DNA repair enzymes against accumulation of 8-oxo-guanine in the spinal motor neurons of amyotrophic lateral sclerosis. (11904761)
2002
42
Tongue and limb myokymia in amyotrophic lateral sclerosis. (10563654)
1999
43
Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis. (9930742)
1999
44
Refined mapping and characterization of the recessive familial amyotrophic lateral sclerosis locus (ALS2) on chromosome 2q33. (9933298)
1998
45
Prognosis in familial amyotrophic lateral sclerosis: progression and survival in patients with glu100gly and ala4val mutations in Cu,Zn superoxide dismutase. (9008494)
1997
46
Apolipoprotein E genotyping in sporadic amyotrophic lateral sclerosis: evidence for a major influence on the clinical presentation and prognosis. (8899655)
1996
47
The glial glutamate transporter complementary DNA in patients with amyotrophic lateral sclerosis. (8797537)
1996
48
Autopsy-proven amyotrophic lateral sclerosis, WaldenstrAPm's macroglobulinemia, and antibodies to sulfated glucuronic acid paragloboside. (7723980)
1995
49
Variants of the heavy neurofilament subunit are associated with the development of amyotrophic lateral sclerosis. (7849698)
1994
50
Insulin-like and fibroblast growth factors in spinal cords, nerve roots and skeletal muscle of human controls and patients with amyotrophic lateral sclerosis. (8017177)
1994

Genetic Variations for Lateral Sclerosis

Expression for genes affiliated with Lateral Sclerosis

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Lateral Sclerosis

Search GEO for disease gene expression data for Lateral Sclerosis.

Pathways for genes affiliated with Lateral Sclerosis

Sources:
52QIAGEN, 30KEGG
See all sources

Pathways related to Lateral Sclerosis according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
11.1ALS2, PRPH, NEFH, SOD1

Compounds for genes affiliated with Lateral Sclerosis

GO Terms for genes affiliated with Lateral Sclerosis

Sources:
16Gene Ontology
See all sources

Cellular components related to Lateral Sclerosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1neurofilamentGO:00588310.8PRPH, NEFH

Biological processes related to Lateral Sclerosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cell deathGO:00821911.4ALS2, NEFH, C9orf72, FIG4, TRPM7, SETX
2locomotory behaviorGO:00762610.9ALS2, FIG4, SOD1
3neurofilament cytoskeleton organizationGO:06005210.5NEFH, SOD1

Products for genes affiliated with Lateral Sclerosis

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Sources for Lateral Sclerosis

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