MCID: LTR001
MIFTS: 58

Lateral Sclerosis

Categories: Genetic diseases, Mental diseases, Muscle diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Lateral Sclerosis

MalaCards integrated aliases for Lateral Sclerosis:

Name: Lateral Sclerosis 12 15 71
Primary Lateral Sclerosis 12 74 20 53 54
Adult-Onset Primary Lateral Sclerosis 12 20
Motor Neuron Disease 44 71
Adult-Onset Pls 20

Classifications:



External Ids:

Disease Ontology 12 DOID:230
ICD9CM 34 335.24
MeSH 44 D016472
NCIt 50 C129933
SNOMED-CT 67 81211007
ICD10 32 G12.23
UMLS 71 C0085084 C0154682

Summaries for Lateral Sclerosis

NINDS : 53 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.  It occurs when nerve cells in the motor regions of the cerebral cortex (the thin layer of cells covering the brain which is responsible for most higher level mental functions) gradually degenerate, causing movements to be slow and effortful.  The disorder often affects the legs first, followed by the body, trunk, arms and hands, and, finally the bulbar muscles (muscles that control speech, swallowing, and chewing).  Symptoms include weakness, muscle stiffness and spasticity, clumsiness, slowing of movement, and problems with balance and speech. PLS is more common in men than in women, with a varied gradual onset that generally occurs between ages 40 and 60. PLS progresses gradually over a number of years, or even decades. Scientists do not believe PLS has a simple hereditary cause.  The diagnosis of PLS requires extensive testing to exclude other diseases. When symptoms begin, PLS may be mistaken for amyotrophic lateral sclerosis (ALS) or spastic paraplegia.  Most neurologists follow an affected individual's clinical course for at least 3 to 4 years before making a diagnosis of PLS.

MalaCards based summary : Lateral Sclerosis, also known as primary lateral sclerosis, is related to amyotrophic lateral sclerosis-parkinsonism/dementia complex 1 and frontotemporal dementia and/or amyotrophic lateral sclerosis 7, and has symptoms including ataxia, myoclonus and hemiplegia. An important gene associated with Lateral Sclerosis is ALS2 (Alsin Rho Guanine Nucleotide Exchange Factor ALS2), and among its related pathways/superpathways are Pathways of neurodegeneration - multiple diseases and Neuroscience. The drugs Mexiletine and Olanzapine have been mentioned in the context of this disorder. Affiliated tissues include spinal cord, brain and cortex, and related phenotypes are babinski sign and abnormal upper motor neuron morphology

Disease Ontology : 12 A motor neuron disease characterized by painless but progressive weakness and stiffness of the muscles of the legs.

GARD : 20 Primary lateral sclerosis (PLS) is a rare neuromuscular disease that affects the nerve cells that control the voluntary muscles. Problems in the legs (such as weakness, stiffness, spasticity, and balance problems) are often observed first, but hand clumsiness and changes in speech can be early symptoms, as well. The condition is progressive (gradually becomes worse over time); however, affected people have a normal life expectancy. The underlying cause of adult PLS is currently unknown. In most cases, it occurs sporadically in people with no family history of the condition. A subtype of PLS, called juvenile PLS, is caused by changes (mutations) in the ALS2 gene and is inherited in an autosomal recessive manner. Treatment is based on the signs and symptoms present in each person.

Wikipedia : 74 Primary lateral sclerosis (PLS) is a very rare neuromuscular disease characterized by progressive muscle... more...

Related Diseases for Lateral Sclerosis

Diseases in the Lateral Sclerosis family:

Primary Lateral Sclerosis, Juvenile Primary Lateral Sclerosis, Adult, 1

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

(show top 50) (show all 935)
# Related Disease Score Top Affiliating Genes
1 amyotrophic lateral sclerosis-parkinsonism/dementia complex 1 33.4 TARDBP SOD1 C9orf72
2 frontotemporal dementia and/or amyotrophic lateral sclerosis 7 33.2 VCP TARDBP FUS
3 frontotemporal dementia and/or amyotrophic lateral sclerosis 3 33.1 TARDBP PRPH C9orf72
4 frontotemporal dementia and/or amyotrophic lateral sclerosis 4 33.1 TARDBP PRPH
5 amyotrophic lateral sclerosis 6 with or without frontotemporal dementia 33.1 VCP FUS
6 frontotemporal dementia and/or amyotrophic lateral sclerosis 2 33.1 PRPH FUS CHCHD10 C9orf72
7 amyotrophic lateral sclerosis 19 32.9 UBQLN2 TARDBP SOD1 PRPH ALS2
8 juvenile amyotrophic lateral sclerosis 32.9 SPG11 SIGMAR1 SETX FUS ALS2
9 amyotrophic lateral sclerosis 20 32.8 SOD1 PRPH HNRNPA1 FIG4 ALS2
10 amyotrophic lateral sclerosis type 5 32.8 VAPB SPG11 SOD1 SETX ANG ALS2
11 amyotrophic lateral sclerosis type 22 32.8 TARDBP PRPH FUS C9orf72
12 neuronopathy, distal hereditary motor, type viib 32.8 TARDBP DCTN1 ALS2
13 amyotrophic lateral sclerosis 11 32.7 VAPB TARDBP SPG11 SETX OPTN FUS
14 amyotrophic lateral sclerosis 7 32.7 VAPB SPG11 SETX PRPH ANG ALS2
15 amyotrophic lateral sclerosis 21 32.7 VAPB SETX PRPH FUS FIG4 ATXN2
16 amyotrophic lateral sclerosis 18 32.7 VAPB UBQLN2 SIGMAR1 SETX PRPH FUS
17 amyotrophic lateral sclerosis 9 32.5 VAPB SPG11 SOD1 SIGMAR1 SETX OPTN
18 amyotrophic lateral sclerosis 16, juvenile 32.4 VCP VAPB UBQLN2 SPG11 SIGMAR1 SETX
19 amyotrophic lateral sclerosis 10 with or without frontotemporal dementia 32.4 VAPB UBQLN2 TARDBP SPG11 SOD1 SETX
20 amyotrophic lateral sclerosis 4, juvenile 32.3 VAPB UBQLN2 TARDBP SPG11 SOD1 SETX
21 perry syndrome 32.3 VCP TARDBP DCTN1 C9orf72
22 spinocerebellar ataxia 2 32.3 VAPB UBQLN2 TARDBP SPG11 SOD1 SETX
23 inclusion body myopathy with paget disease of bone and frontotemporal dementia 32.2 VCP UBQLN2 TARDBP HNRNPA1 FUS C9orf72
24 amyotrophic lateral sclerosis type 15 32.2 VAPB UBQLN2 SPG11 SIGMAR1 SETX PRPH
25 amyotrophic lateral sclerosis type 14 32.2 VCP VAPB UBQLN2 SPG11 SIGMAR1 SETX
26 proximal spinal muscular atrophy 32.2 VAPB SETX
27 distal hereditary motor neuropathies 32.1 SETX DCTN1
28 amyotrophic lateral sclerosis 8 32.1 VCP VAPB UBQLN2 TARDBP SPG11 SOD1
29 frontotemporal dementia 32.0 VCP UBQLN2 TARDBP SOD1 OPTN NEFH
30 amyotrophic lateral sclerosis type 12 32.0 VAPB UBQLN2 TARDBP SPG11 SOD1 SIGMAR1
31 amyotrophic lateral sclerosis 1 32.0 VCP VAPB UBQLN2 TARDBP SPG11 SOD1
32 neuromuscular disease 32.0 VCP SOD1 NEFH FUS FIG4 DCTN1
33 progressive bulbar palsy 31.9 VAPB SOD1 SETX NEFH FUS C9orf72
34 spinal muscular atrophy, distal, autosomal recessive, 4 31.9 UBQLN2 TARDBP SPG11 SOD1 SETX OPTN
35 amyotrophic lateral sclerosis type 6 31.8 VCP VAPB UBQLN2 TARDBP SPG11 SOD1
36 progressive muscular atrophy 31.8 VCP VAPB UBQLN2 TARDBP SOD1 OPTN
37 pick disease of brain 31.8 VCP UBQLN2 TARDBP SOD1 OPTN NEFH
38 multisystem proteinopathy 31.8 VCP UBQLN2 TARDBP OPTN HNRNPA1 FUS
39 frontotemporal dementia and/or amyotrophic lateral sclerosis 1 31.7 VCP VAPB UBQLN2 TARDBP SPG11 SOD1
40 motor neuron disease 31.5 VCP VAPB UBQLN2 TARDBP SOD1 SIGMAR1
41 dementia 31.4 VCP UBQLN2 TARDBP HNRNPA1 FUS CHCHD10
42 muscular atrophy 31.4 VAPB TARDBP SIGMAR1 SETX HNRNPA1 FUS
43 aphasia 31.2 VCP TARDBP OPTN FUS C9orf72
44 neuropathy 31.1 SPG11 SOD1 SETX OPTN NEFH FIG4
45 alzheimer disease 31.0 VCP TARDBP SOD1 SIGMAR1 OPTN NEFH
46 spinal muscular atrophy 31.0 VAPB TARDBP SOD1 SIGMAR1 SETX HNRNPA1
47 semantic dementia 31.0 TARDBP C9orf72
48 dysgraphia 30.9 TARDBP FUS C9orf72
49 supranuclear palsy, progressive, 1 30.9 VCP TARDBP SOD1 FUS DCTN1 C9orf72
50 agraphia 30.9 VCP TARDBP C9orf72

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



Diseases related to Lateral Sclerosis

Symptoms & Phenotypes for Lateral Sclerosis

Human phenotypes related to Lateral Sclerosis:

31 (show all 12)
# Description HPO Frequency HPO Source Accession
1 babinski sign 31 hallmark (90%) HP:0003487
2 abnormal upper motor neuron morphology 31 hallmark (90%) HP:0002127
3 generalized hyperreflexia 31 hallmark (90%) HP:0007034
4 dysphagia 31 frequent (33%) HP:0002015
5 loss of speech 31 frequent (33%) HP:0002371
6 spastic gait 31 frequent (33%) HP:0002064
7 emg: chronic denervation signs 31 frequent (33%) HP:0003444
8 spastic dysarthria 31 frequent (33%) HP:0002464
9 pseudobulbar signs 31 frequent (33%) HP:0002200
10 progressive spastic paraparesis 31 frequent (33%) HP:0007199
11 motor axonal neuropathy 31 occasional (7.5%) HP:0007002
12 cervical spinal cord atrophy 31 occasional (7.5%) HP:0010873

UMLS symptoms related to Lateral Sclerosis:


ataxia, myoclonus, hemiplegia, muscular fasciculation

GenomeRNAi Phenotypes related to Lateral Sclerosis according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00106-A-0 9.74 DCTN1
2 Decreased viability GR00221-A-2 9.74 SOD1
3 Decreased viability GR00221-A-3 9.74 SOD1
4 Decreased viability GR00221-A-4 9.74 FUS SOD1
5 Decreased viability GR00249-S 9.74 ANG DCTN1 OPTN SIGMAR1 SOD1 UBQLN2
6 Decreased viability GR00381-A-1 9.74 ANG FIG4 SIGMAR1 VCP
7 Decreased viability GR00381-A-3 9.74 SIGMAR1
8 Decreased viability GR00386-A-1 9.74 OPTN SETX SIGMAR1 UBQLN2
9 Decreased viability GR00402-S-2 9.74 CHCHD10 NEFH SETX UBQLN2 VAPB VCP

MGI Mouse Phenotypes related to Lateral Sclerosis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.17 ALS2 ATXN2 C9orf72 CHCHD10 DCTN1 FIG4
2 cellular MP:0005384 10 ANG C9orf72 CHCHD10 DCTN1 NEFH SETX
3 immune system MP:0005387 9.9 ALS2 ANG C9orf72 CHCHD10 DCTN1 FIG4
4 muscle MP:0005369 9.56 ALS2 CHCHD10 DCTN1 FIG4 SOD1 TARDBP
5 nervous system MP:0003631 9.44 ALS2 ATXN2 C9orf72 CHCHD10 DCTN1 FIG4

Drugs & Therapeutics for Lateral Sclerosis

Drugs for Lateral Sclerosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 328)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Mexiletine Approved, Investigational Phase 4 31828-71-4 4178
2
Olanzapine Approved, Investigational Phase 2, Phase 3 132539-06-1 4585 135398745
3
Hydroxocobalamin Approved Phase 2, Phase 3 13422-51-0 11953898 15589840
4
Methylcobalamin Approved, Investigational Phase 2, Phase 3 13422-55-4
5
Guaifenesin Approved, Investigational, Vet_approved Phase 3 93-14-1 3516
6
Minocycline Approved, Investigational Phase 3 10118-90-8 5281021
7
Ceftriaxone Approved Phase 3 73384-59-5 5479530 5361919
8
Valproic acid Approved, Investigational Phase 3 99-66-1 3121
9
Mecasermin Approved, Investigational Phase 3 68562-41-4
10
Acetylcholine Approved, Investigational Phase 2, Phase 3 51-84-3 187
11
Dextromethorphan Approved Phase 3 125-71-3 5360696 5362449
12
Quinidine Approved, Investigational Phase 3 56-54-2 441074
13
Lenograstim Approved, Investigational Phase 2, Phase 3 135968-09-1
14
Sargramostim Approved, Investigational Phase 2, Phase 3 123774-72-1, 83869-56-1
15
Citalopram Approved Phase 3 59729-33-8 2771
16
Memantine Approved, Investigational Phase 2, Phase 3 19982-08-2 4054
17
Edaravone Approved, Investigational Phase 3 89-25-8 70335
18
Trazodone Approved, Investigational Phase 2, Phase 3 19794-93-5 5533
19
Iron Approved Phase 2, Phase 3 7439-89-6 23925 29936
20
Deferiprone Approved Phase 2, Phase 3 30652-11-0 2972
21
Ravulizumab Approved, Investigational Phase 3 1803171-55-2
22
Sodium citrate Approved, Investigational Phase 3 68-04-2
23
Coal tar Approved Phase 2, Phase 3 8007-45-2
24
Dopamine Approved Phase 3 51-61-6, 62-31-7 681
25
Pramipexole Approved, Investigational Phase 3 104632-26-0 59868 119570
26
Lithium carbonate Approved Phase 2, Phase 3 554-13-2
27
Cyanocobalamin Approved, Nutraceutical Phase 2, Phase 3 68-19-9 44176380
28
Coenzyme Q10 Approved, Investigational, Nutraceutical Phase 3 303-98-0 5281915
29
Tyrosine Approved, Investigational, Nutraceutical Phase 3 60-18-4 6057
30
Citric acid Approved, Nutraceutical, Vet_approved Phase 3 77-92-9 311
31
Cobalamin Experimental Phase 2, Phase 3 13408-78-1 6857388
32 Nabiximols Investigational Phase 2, Phase 3 56575-23-6
33
Simendan Investigational Phase 3 131741-08-7
34 Molgramostim Investigational Phase 2, Phase 3 99283-10-0
35
Ibudilast Investigational Phase 2, Phase 3 50847-11-5 3671
36
Tauroursodeoxycholic acid Experimental, Investigational Phase 2, Phase 3 14605-22-2 12443252
37 Vitamin B 12 Phase 2, Phase 3
38 Vitamin B12 Phase 2, Phase 3
39 Neurotransmitter Agents Phase 3
40 Excitatory Amino Acid Antagonists Phase 3
41 Immunoglobulin G Phase 3
42 gamma-Globulins Phase 3
43 Immunoglobulins, Intravenous Phase 3
44 Rho(D) Immune Globulin Phase 3
45 Diuretics, Potassium Sparing Phase 3
46 Sodium Channel Blockers Phase 3
47 Anti-Arrhythmia Agents Phase 3
48 Cardiotonic Agents Phase 3
49 Phosphodiesterase 3 Inhibitors Phase 3
50 Anti-Infective Agents Phase 3

Interventional clinical trials:

(show top 50) (show all 594)
# Name Status NCT ID Phase Drugs
1 Role of Non-invasive Ventilation in Amyotrophic Lateral Sclerosis: Volume Versus Pressure Mode Unknown status NCT00560287 Phase 4
2 Care (Canadian ALS Riluzole Evaluation) Multicentre Phase IV Comparative Study of the Effects of Riluzole 50mg Bid on the Survival of ALS Subjects Compared to Historical Controls Completed NCT00542412 Phase 4 Riluzole
3 Feasibility of Telesurveillance and Home Cough Assistance for Amyotrophic Lateral Patients (ALS) Completed NCT00613899 Phase 4
4 Mexiletine for the Treatment of Muscle Cramps in ALS Completed NCT01811355 Phase 4 Mexiletine;Placebo
5 Japanese Early-stage Clinical Trial of Ultra-high Dose Methylcobalamin for Amyotrophic Lateral Sclerosis: a Pivotal Phase 3 Randomized Controlled Study Unknown status NCT03548311 Phase 3 methylcobalamin;saline solution
6 Randomized, Placebo-controlled Parallel Group Study for the Evaluation of an Oral Dose of 10mg Olanzapine in Combination With Riluzole for the Treatment of Loss of Appetite in Patients With Amyotrophic Lateral Sclerosis (ALS) Unknown status NCT00876772 Phase 2, Phase 3 Olanzapine
7 A Phase III, Multi-Center, Double-Blind, Placebo Controlled, Randomized Study of Creatine Monohydrate in Patients With Amyotrophic Lateral Sclerosis Unknown status NCT00069186 Phase 3 Creatine Monohydrate
8 Noninvasive Ventilation in ALS Patients With Mild Respiratory Involvement Completed NCT00386464 Phase 2, Phase 3
9 Phase 2-3 - Memantine for Disability in Amyotrophic Lateral Sclerosis Completed NCT00353665 Phase 2, Phase 3 Memantine (Ebixa);riluzole;Placebo
10 A Fase II, Randomized, Double-Blind, Placebo-Controlled, Multicentre Study for the Safety and Efficacy on Spasticity Symptoms of a Cannabis Sativa Extract in Motor Neuron Disease Patients Completed NCT01776970 Phase 2, Phase 3 Cannabis Sativa extract Oromucosal spray
11 A European, Randomised, Double-blind, Active Comparator Controlled, Cross-over, Efficacy and Safety Study of a New 10% Ready To-use Liquid Human Intravenous Immunoglobulin (I10E) Versus Kiovig® in Patients With Multifocal Motor Neuropathy Completed NCT01951924 Phase 3 Biological : I10E (Human normal Immunoglobulin for intravenous administration 100mg/mL);Biological: Kiovig® (Human normal Immunoglobulin for intravenous administration 100mg/mL)
12 Effects of Oral Levosimendan (ODM-109) on Respiratory Function in Patients With ALS Completed NCT03505021 Phase 3 Levosimendan;Placebo for levosimendan
13 A Phase 3, Randomised, Placebo-Controlled Trial of Arimoclomol in Amyotropic Lateral Sclerosis Completed NCT03491462 Phase 3 Arimoclomol;Placebo oral capsule
14 Study of Dopamine and Serotonin Transporters in Patients With Amyotrophic Lateral Sclerosis and Controls. Analysis With 123I-FP-CIT (Datscan) and 123I-ADAM Brain SPECT Completed NCT01160263 Phase 3 SPECT : 123 I-FP-CIT (DATSCAN) and 123I-ADAM
15 Randomized Crossover Design Trial of Vitamin E vs Placebo for Treatment of Cramps in Amyotrophic Lateral Sclerosis. Completed NCT00372879 Phase 3
16 Clinical Trial Ceftriaxone in Subjects With Amyotrophic Lateral Sclerosis (ALS) Completed NCT00349622 Phase 3 ceftriaxone
17 A Confirmatory Study of MCI-186 for Treatment of Amyotrophic Lateral Sclerosis in Double-blind, Parallel-group, Placebo-controlled Manner. Completed NCT00330681 Phase 3 MCI-186;Placebo of MCI-186
18 A Randomized, Double-Blind, Placebo-Controlled Sequential Clinical Trial of Sodium Valproate in ALS Completed NCT00136110 Phase 3 Sodium Valproate
19 Minocycline to Treat Amyotrophic Lateral Sclerosis Completed NCT00047723 Phase 3 minocycline
20 Insulin-like Growth Factor-1 in Amyotrophic Lateral Sclerosis (ALS) Completed NCT00035815 Phase 3 Insulin like growth factor, type 1;Placebo
21 Multicenter, Randomised, Double-blind, Placebo-controlled, Parallel Group, Phase 2/3 Study to Compare the Efficacy and Safety of Masitinib Completed NCT02588677 Phase 2, Phase 3 Masitinib (4.5);Riluzole;Placebo;Masitinib (3.0)
22 Phase II/III Randomized, Placebo-Controlled Trial of Arimoclomol in SOD1 Positive Familial Amyotrophic Lateral Sclerosis (ALS) Completed NCT00706147 Phase 2, Phase 3 Arimoclomol;Placebo
23 An Open-label Safety Extension Study of TRO19622 in Amyotrophic Lateral Sclerosis (ALS) Patients Treated With Riluzole Completed NCT01285583 Phase 2, Phase 3 TRO19622
24 A Randomized, Double-Blind, Placebo-Controlled, Multi-Center Study of the Safety and Efficacy of Dexpramipexole in Subjects With Amyotrophic Lateral Sclerosis Completed NCT01281189 Phase 3 Dexpramipexole;Placebo
25 Effect of Intrathecal Administration of Hematopoietic Stem Cells in Patients With Amyotrophic Lateral Sclerosis (ALS) Completed NCT01933321 Phase 2, Phase 3
26 A Long-Term Study in Patients With Amyotrophic Lateral Sclerosis (ALS) Completed NCT00445172 Phase 2, Phase 3 E0302 (mecobalamin)
27 An Expanded Controlled Study of MCI-186 for Treatment of Amyotrophic Lateral Sclerosis in Double-Blind, Parallel-Group, Placebo-Controlled Manner (Phase 3) Completed NCT00424463 Phase 3 MCI-186;Placebo of MCI-186
28 A Randomized, Double-Blind, Placebo-Controlled Study of Safety and Efficacy of Botulinum Toxin Type B (Myobloc) in Sialorrhea in Amyotrophic Lateral Sclerosis Completed NCT00125203 Phase 2, Phase 3 Botulinum toxin type B (Myobloc)
29 A Double-Blind Controlled, Multicenter Phase II/III Study to Assess the Safety and Efficacy of AVP-923 (Dextromethorphan/Quinidine) in the Treatment of Pseudobulbar Affect in Patients With Amyotrophic Lateral Sclerosis Completed NCT00021697 Phase 3 AVP-923
30 The Effect of Granulocyte Colony Stimulating Factor (GCSF) in the Treatment of Amyotrophic Lateral Sclerosis (ALS) Patients Referred to Tehran Imam Khomeini and Shariati Hospital Centers in 2013 Completed NCT01825551 Phase 2, Phase 3 Granulocyte Colony Stimulating Factor;Placebo
31 An Exploratory Study of MCI-186 for Treatment of Amyotrophic Lateral Sclerosis (Severity Classification III) in Double-Blind, Parallel-Group, Placebo-Controlled Manner Completed NCT00415519 Phase 3 MCI-186;Placebo of MCI-186
32 A Phase II/III Study in Patients With Amyotrophic Lateral Sclerosis (ALS) Completed NCT00444613 Phase 2, Phase 3 E0302 (mecobalamin);E0302 (mecobalamin);Placebo
33 Efficacy and Safety Study of MCI-186 for Treatment of the Patients With Amyotrophic Lateral Sclerosis (ALS) 2 Completed NCT01492686 Phase 3 MCI-186;Placebo;MCI-186 in open label phase
34 A Phase 3, Randomized Double-Blind, Placebo-Controlled Multicenter Study to Evaluate Efficacy and Safety of Repeated Administration of NurOwn® (Autologous Mesenchymal Stem Cells Secreting Neurotrophic Factors) in Participants With ALS Completed NCT03280056 Phase 3
35 Effects of Oral Levosimendan (ODM-109) on Respiratory Function in Patients With ALS: Open-Label Extension for Patients Completing Study 3119002 Completed NCT03948178 Phase 3 Levosimendan
36 Repetitive Transcranial Magnetic Stimulation in Amyotrophic Lateral Sclerosis Completed NCT00833820 Phase 2, Phase 3
37 Evaluation of the Safety, Tolerability, Efficacy and Activity of AMX0035, a Fixed Combination of Phenylbutyrate (PB) and Tauroursodeoxycholic Acid (TUDCA), for the Treatment of ALS Completed NCT03127514 Phase 2, Phase 3 AMX0035
38 A Phase 3, Open-Label Extension Study of Tirasemtiv for Patients With Amyotrophic Lateral Sclerosis (ALS) Who Completed VITALITY-ALS (CY 4031) Completed NCT02936635 Phase 3 tirasemtiv
39 Phase II/III, Multicenter, Randomized, Parallel Group, Double-blind, Placebo Controlled Study to Assess Safety and Efficacy of TRO19622 in Amyotrophic Lateral Sclerosis (ALS) Patients Treated With Riluzole Completed NCT00868166 Phase 3 Olesoxime;Placebo Comparator;Riluzole
40 A Phase 3, Multi-National, Double-Blind, Randomized, Placebo-Controlled, Stratified, Parallel Group, Study to Evaluate the Safety, Tolerability and Efficacy of Tirasemtiv in Patients With Amyotrophic Lateral Sclerosis (ALS) Completed NCT02496767 Phase 3 Tirasemtiv;Placebo tablets
41 An Open-label, 8- Week, Flexible Dose Trial of Escitalopram (Lexapro®) in Comorbid Major Depression With Amyotrophic Lateral Sclerosis and Multiple Sclerosis Completed NCT00965497 Phase 3 escitalopram
42 A Double-Blind, Randomized, Placebo-Controlled, Multicenter Study to Assess the Safety and Efficacy and to Determine the Pharmacokinetics of Two Doses of AVP-923 (Dextromethorphan/Quinidine) in the Treatment of Pseudobulbar Affect (PBA) in Patients With Amyotrophic Lateral Sclerosis (ALS) and Multiple Sclerosis (MS) Completed NCT00573443 Phase 3 dextromethorphan hydrobromide 20 mg and quinidine sulfate 10 mg;dextromethorphan hydrobromide 30 mg and quinidine sulfate 10 mg;Placebo
43 Motor Neurone Disease - Systematic Multi-Arm Adaptive Randomised Trial Recruiting NCT04302870 Phase 2, Phase 3 Memantine Hydrochloride Oral Solution;Trazodone Hydrochloride oral solution;Placebo oral solution
44 A Multicenter, Randomized, Double-Blind, Placebo-Controlled Phase 2 Study of Cu(II)ATSM in Patients With Amyotrophic Lateral Sclerosis/Motor Neuron Disease Recruiting NCT04082832 Phase 2, Phase 3 Cu(II)ATSM;Placebos
45 A Randomised, Double-blind, Single-centre Study on the Safety, Tolerability and Efficacy of Cannabis Based Medicine Extract (MediCabilis CBD Oil) in Slowing the Disease Progression in Amyotrophic Lateral Sclerosis or Motor Neurone Disease Patients Recruiting NCT03690791 Phase 3 MediCabilis CBD Oil;Placebo Oil
46 Conservative Iron Chelation as a Disease-modifying Strategy in Amyotrophic Lateral Sclerosis: Multicentre, Parallel-group, Placebo-controlled, Randomized Clinical Trial of Deferiprone Recruiting NCT03293069 Phase 2, Phase 3 Deferiprone;Placebo Oral Tablet
47 A Phase 3, Multi-center, Open-label, Safety Extension Study of Oral Edaravone Administered Over 96 Weeks in Subjects With Amyotrophic Lateral Sclerosis (ALS) Recruiting NCT04577404 Phase 3 MT-1186
48 A Phase 3, Double-Blind, Randomized, Placebo-Controlled, Parallel Group, Multicenter Study With an Open-Label Extension to Evaluate the Efficacy and Safety of Ravulizumab in Patients With Amyotrophic Lateral Sclerosis (ALS) Recruiting NCT04248465 Phase 3 Placebo
49 Safety and Efficacy of Tauroursodeoxycholic (TUDCA) as add-on Treatment in Patients Affected by Amyotrophic Lateral Sclerosis (ALS) Recruiting NCT03800524 Phase 3 Tauroursodeoxycholic Acid;Placebo
50 HEALEY ALS Platform Trial Recruiting NCT04297683 Phase 2, Phase 3 Zilucoplan;Verdiperstat;CNM-Au8;Pridopidine

Search NIH Clinical Center for Lateral Sclerosis

Cochrane evidence based reviews: motor neuron disease

Genetic Tests for Lateral Sclerosis

Anatomical Context for Lateral Sclerosis

MalaCards organs/tissues related to Lateral Sclerosis:

40
Spinal Cord, Brain, Cortex, Bone Marrow, Skeletal Muscle, Skin, Bone

Publications for Lateral Sclerosis

Articles related to Lateral Sclerosis:

(show top 50) (show all 24356)
# Title Authors PMID Year
1
Astrocytic protection of spinal motor neurons but not cortical neurons against loss of Als2/alsin function. 61 54
19304783 2009
2
A novel ALS2 splice-site mutation in a Cypriot juvenile-onset primary lateral sclerosis family. 61 54
19122027 2009
3
SOD1-associated ALS: a promising system for elucidating the origin of protein-misfolding disease. 54 61
19436494 2008
4
Novel homozygous ALS2 nonsense mutation (p.Gln715X) in sibs with infantile-onset ascending spastic paralysis: the first cases from northwestern Europe. 61 54
18523452 2008
5
A strategy for developing effective amyotropic lateral sclerosis pharmacotherapy: from clinical trials to novel pharmacotherapeutic strategies. 61 54
18627324 2008
6
Spastin mutations in sporadic adult-onset upper motor neuron syndromes. 54 61
16240363 2005
7
Mitochondrial proteomic analysis of a cell line model of familial amyotrophic lateral sclerosis. 61 54
15501831 2004
8
Folding of human superoxide dismutase: disulfide reduction prevents dimerization and produces marginally stable monomers. 54 61
15522970 2004
9
An intersubunit disulfide bond prevents in vitro aggregation of a superoxide dismutase-1 mutant linked to familial amytrophic lateral sclerosis. 54 61
15109247 2004
10
17q-linked frontotemporal dementia-amyotrophic lateral sclerosis without tau mutations with tau and alpha-synuclein inclusions. 54 61
15023818 2004
11
Relevance of oxidative injury in the pathogenesis of motor neuron diseases. 54 61
14753657 2003
12
Unstable mutants in the peripheral endosomal membrane component ALS2 cause early-onset motor neuron disease. 54 61
14668431 2003
13
The first nonsense mutation in alsin results in a homogeneous phenotype of infantile-onset ascending spastic paralysis with bulbar involvement in two siblings. 54 61
12919135 2003
14
ALS2, a novel guanine nucleotide exchange factor for the small GTPase Rab5, is implicated in endosomal dynamics. 54 61
12837691 2003
15
Infantile ascending hereditary spastic paralysis (IAHSP): clinical features in 11 families. 54 61
12601111 2003
16
[Tauopathies--a new class of neurodegenerative diseases]. 54 61
11256158 2001
17
Motor neuron-astrocyte interactions and levels of Cu,Zn superoxide dismutase in sporadic amyotrophic lateral sclerosis. 54 61
7895821 1995
18
Considerations on the concept, definition, and diagnosis of amyotrophic lateral sclerosis. 61
33510061 2021
19
Phenotypic heterogeneity in amyotrophic lateral sclerosis type 8 and modifying mechanisms of neurodegeneration. 61
33510073 2021
20
Regulation of neuronal bioenergetics as a therapeutic strategy in neurodegenerative diseases. 61
33433460 2021
21
Suicide transport blockade of motor neuron survival generates a focal graded injury and functional deficit. 61
33318406 2021
22
Backdoor intrusion: retrotoxicity can explain targeted motor neuron death in amyotrophic lateral sclerosis. 61
33318449 2021
23
Complement: a global immunometabolic regulator in amyotrophic lateral sclerosis. 61
33269780 2021
24
Degenerative and regenerative processes in amyotrophic lateral sclerosis: motor reserve, adaptation and putative compensatory changes. 61
33269779 2021
25
The important functional role of TDP-43 plays in amyotrophic lateral sclerosis-frontotemporal dementia. 61
33063724 2021
26
Stem cell-derived extracellular vesicles as potential mechanism for repair of microvascular damage within and outside of the central nervous system in amyotrophic lateral sclerosis: perspective schema. 61
33063723 2021
27
Creatine kinase in the diagnosis and prognostic prediction of amyotrophic lateral sclerosis: a retrospective case-control study. 61
32985493 2021
28
Loss-of-function variants in NEK1 are associated with an increased risk of sporadic ALS in the Japanese population. 61
32920598 2021
29
Some pathogenic SETX variants are partially conserved during evolution. 61
33333218 2021
30
Transport Alteration of 4-Phenyl Butyric Acid Mediated by a Sodium- and Proton-Coupled Monocarboxylic Acid Transporter System in ALS Model Cell Lines (NSC-34) Under Inflammatory States. 61
33098824 2021
31
Pneumomediastinum while using mechanical insufflation-exsufflation after recovery from riluzole-induced interstitial lung disease. 61
33598572 2021
32
Progress in progestin-based therapies for neurological disorders. 61
33359391 2021
33
D-amino acid oxidase (DAO) rare genetic missense variant p.Pro103Leu and gastric cancer. 61
33604048 2021
34
Which are the factors influencing NIV adaptation and tolerance in ALS patients? 61
32710206 2021
35
Amide signal intensities may be reduced in the motor cortex and the corticospinal tract of ALS patients. 61
32909054 2021
36
Co-incidental C9orf72 expansion mutation-related frontotemporal lobar degeneration pathology and sporadic Creutzfeldt-Jakob disease. 61
33131137 2021
37
SQSTM1L341V variant that is linked to sporadic ALS exhibits impaired association with MAP1LC3 in cultured cells. 61
33319079 2021
38
Delayed onset of inherited ALS by deletion of the BDNF receptor TrkB.T1 is non-cell autonomous. 61
33359475 2021
39
The Peptidyl-prolyl Isomerase Pin1 in Neuronal Signaling: from Neurodevelopment to Neurodegeneration. 61
33083964 2021
40
Anti-IgLON5 Disease: A New Bulbar-Onset Motor Neuron Mimic Syndrome. 61
33531378 2021
41
Molecular imaging for mitochondrial metabolism and oxidative stress in mitochondrial diseases and neurodegenerative disorders. 61
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42
Visualization of Motor Cortex Involvement by 18F-THK5351 PET Potentially Strengthens Diagnosis of Amyotrophic Lateral Sclerosis. 61
33323735 2021
43
Basilar invagination: A mimicker of bulbar-onset amyotrophic lateral sclerosis. 61
33553704 2021
44
A susceptibility-weighted imaging qualitative score of the motor cortex may be a useful tool for distinguishing clinical phenotypes in amyotrophic lateral sclerosis. 61
32886203 2021
45
TDP-43-specific Autoantibody Decline in Patients With Amyotrophic Lateral Sclerosis. 61
33361387 2021
46
Impact of COVID- 19 Infection Among Hospitalized Amyotrophic Lateral Sclerosis Patients. 61
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47
BDNF-overexpressing human umbilical cord mesenchymal stem cell-derived motor neurons improve motor function and prolong survival in amyotrophic lateral sclerosis mice. 61
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48
Brain metabolic correlates of apathy in amyotrophic lateral sclerosis: An 18F-FDG-positron emission tomography stud. 61
33175462 2021
49
Retraction Notice to "Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis" [Neurobiology of Disease, 30/3 (June 2008) 400 - 407]. 61
33371961 2021
50
Broadening the clinical spectrum of FUS mutations: a case with monomelic amyotrophy with a late progression to amyotrophic lateral sclerosis. 61
33001405 2021

Variations for Lateral Sclerosis

Expression for Lateral Sclerosis

Search GEO for disease gene expression data for Lateral Sclerosis.

Pathways for Lateral Sclerosis

Pathways related to Lateral Sclerosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.8 VCP VAPB UBQLN2 TARDBP SPG11 SOD1
2 12.17 TARDBP SOD1 SIGMAR1 OPTN NEFH DCTN1
3
Show member pathways
11.47 PRPH NEFH DCTN1
4 10.84 SOD1 PRPH NEFH ALS2

GO Terms for Lateral Sclerosis

Cellular components related to Lateral Sclerosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 nucleus GO:0005634 10.24 VCP UBQLN2 TARDBP SPG11 SOD1 SIGMAR1
2 cytoplasm GO:0005737 10.16 VCP VAPB UBQLN2 TARDBP SPG11 SOD1
3 cell projection GO:0042995 9.95 SPG11 SIGMAR1 SETX PRPH NEFH C9orf72
4 cytoplasmic vesicle GO:0031410 9.87 UBQLN2 SPG11 SOD1 SIGMAR1 OPTN C9orf72
5 neuronal cell body GO:0043025 9.8 SOD1 FUS DCTN1 ANG ALS2
6 lipid droplet GO:0005811 9.63 VCP SIGMAR1 FIG4
7 autophagosome GO:0005776 9.61 UBQLN2 OPTN C9orf72
8 cytoplasmic stress granule GO:0010494 9.46 VCP TARDBP C9orf72 ATXN2
9 growth cone GO:0030426 9.35 SIGMAR1 SETX C9orf72 ANG ALS2
10 axon GO:0030424 9.17 SPG11 SETX PRPH NEFH DCTN1 C9orf72

Biological processes related to Lateral Sclerosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 locomotory behavior GO:0007626 9.43 SOD1 FIG4 ALS2
2 intermediate filament cytoskeleton organization GO:0045104 9.4 PRPH NEFH
3 stress granule assembly GO:0034063 9.37 C9orf72 ATXN2
4 regulation of autophagosome assembly GO:2000785 9.32 UBQLN2 C9orf72
5 autophagy GO:0006914 9.26 VCP UBQLN2 OPTN C9orf72
6 neurofilament cytoskeleton organization GO:0060052 9.16 SOD1 NEFH
7 maintenance of synapse structure GO:0099558 8.62 DCTN1 CHCHD10

Molecular functions related to Lateral Sclerosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.96 VCP VAPB UBQLN2 TARDBP SPG11 SOD1
2 small GTPase binding GO:0031267 9.56 SOD1 OPTN C9orf72 ALS2
3 polyubiquitin modification-dependent protein binding GO:0031593 9.33 VCP UBQLN2 OPTN
4 identical protein binding GO:0042802 9.32 VCP UBQLN2 TARDBP SOD1 SIGMAR1 SETX

Sources for Lateral Sclerosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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