MCID: SYN058
MIFTS: 44

Synucleinopathy

Categories: Neuronal diseases

Aliases & Classifications for Synucleinopathy

MalaCards integrated aliases for Synucleinopathy:

Name: Synucleinopathy 12 15
Alpha Synucleinopathies 12
Synucleinopathies 12

Classifications:



External Ids:

Disease Ontology 12 DOID:0050890

Summaries for Synucleinopathy

Disease Ontology : 12 A neurodegenerative disease that is characterized by the abnormal accumulation of aggregates of alpha-synuclein protein in neurons, nerve fibres or glial cells.

MalaCards based summary : Synucleinopathy, also known as alpha synucleinopathies, is related to parkinson disease, late-onset and dementia, lewy body. An important gene associated with Synucleinopathy is SNCA (Synuclein Alpha), and among its related pathways/superpathways are Neuroscience and Microglia Activation During Neuroinflammation: Overview. The drugs Dopamine and Dopamine Agents have been mentioned in the context of this disorder. Affiliated tissues include brain, eye and olfactory bulb, and related phenotypes are Decreased viability and Decreased viability

Wikipedia : 76 Synucleinopathies (also called α-Synucleinopathies) are neurodegenerative diseases characterised by the... more...

Related Diseases for Synucleinopathy

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

(show top 50) (show all 52)
# Related Disease Score Top Affiliating Genes
1 parkinson disease, late-onset 31.9 APP CALB1 GBA GRM5 KLK6 MAPT
2 dementia, lewy body 31.9 APP GBA MAPT PRKN SNCA SNCAIP
3 multiple system atrophy 1 31.2 MAPT PRKN SNCA SNCB TH
4 dementia 30.8 APP CHMP2B GBA MAPT PRKN SNCA
5 gaucher's disease 30.7 GBA PRKN SNCA
6 dysautonomia 30.5 PRKN SNCA SNCAIP
7 supranuclear palsy, progressive, 1 30.3 APP MAPT PRKN PVALB SNCA TH
8 pick disease of brain 30.3 APP MAPT SNCA
9 rem sleep behavior disorder 10.4
10 hereditary late-onset parkinson disease 10.3 GBA SNCA
11 parkinson disease 10 10.3 PRKN SNCA
12 early-onset parkinson's disease 10.3 GBA PRKN SNCA
13 breast fibrosarcoma 10.3 CALB1 PVALB
14 nominal aphasia 10.3 CHMP2B MAPT
15 primary lateral sclerosis, adult, 1 10.3 MAPT SNCA
16 progressive non-fluent aphasia 10.3 CHMP2B MAPT
17 toxic encephalopathy 10.3 APP SNCA TH
18 neonatal hypoxic and ischemic brain injury 10.3 PVALB TH
19 behavioral variant of frontotemporal dementia 10.3 CHMP2B MAPT
20 neurodegeneration with brain iron accumulation 10.3 SNCA SNCB SNCG
21 alzheimer disease 10.3
22 binswanger's disease 10.3 APP MAPT SST
23 leukoencephalopathy, hereditary diffuse, with spheroids 10.3 APP MAPT SNCA
24 parkinson disease 3, autosomal dominant 10.2 MAPT PRKN SNCA SNCAIP
25 tremor 10.2 GBA MAPT PRKN SNCA
26 spinocerebellar ataxia 37 10.2 CALB1 SNCA
27 familial idiopathic basal ganglia calcification 10.2 APP MAPT
28 aphasia 10.2 APP CHMP2B MAPT SNCA
29 restless legs syndrome 10.2 PRKN SNCA TH
30 aging 10.2
31 pure autonomic failure 10.2
32 frontotemporal dementia 10.2 APP CHMP2B MAPT SNCA
33 semantic dementia 10.2 CHMP2B MAPT
34 nervous system disease 10.2 APP MAPT PRKN SNCA TH
35 kohlschutter-tonz syndrome 10.2 APP MAPT
36 disease of mental health 10.2 APP GRM5 MAPT PVALB SNCA
37 status epilepticus 10.2 CALB1 CALB2 PVALB SST
38 postencephalitic parkinson disease 10.2 MAPT SNCA
39 movement disease 10.2 GBA MAPT PRKN SNCA SNCG TH
40 central nervous system disease 10.1 APP GRM5 MAPT PRKN SNCA TH
41 kluver-bucy syndrome 10.1 APP MAPT
42 creutzfeldt-jakob disease 10.1
43 depression 10.1
44 schizophrenia 9.9
45 neurodegeneration with brain iron accumulation 1 9.9
46 ichthyosis prematurity syndrome 9.9
47 lysosomal storage disease 9.9
48 amyloidosis 9.9
49 primary orthostatic hypotension 9.9
50 dysphagia 9.9

Graphical network of the top 20 diseases related to Synucleinopathy:



Diseases related to Synucleinopathy

Symptoms & Phenotypes for Synucleinopathy

GenomeRNAi Phenotypes related to Synucleinopathy according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00240-S-1 10.05 PVALB SST
2 Decreased viability GR00381-A-1 10.05 GRM5 HSF1 PVALB
3 Decreased viability GR00402-S-2 10.05 APP CALB1 CALB2 CHMP2B GBA GRM5
4 no effect GR00402-S-1 9.62 APP CALB1 CALB2 CHMP2B GBA GRM5

MGI Mouse Phenotypes related to Synucleinopathy:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.13 APP CALB1 CALB2 GBA GRM5 HSF1
2 nervous system MP:0003631 10.06 APP CALB1 CALB2 CHMP2B GBA GRM5
3 integument MP:0010771 9.86 APP GBA GRM5 HSF1 MAPT PRKN
4 no phenotypic analysis MP:0003012 9.61 APP HSF1 MAPT PRKN PVALB SNCA
5 normal MP:0002873 9.36 APP CALB1 CALB2 GBA MAPT PRKN

Drugs & Therapeutics for Synucleinopathy

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

(show all 11)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dopamine Approved Not Applicable 51-61-6, 62-31-7 681
2 Dopamine Agents Not Applicable
3 Dopamine agonists Not Applicable
4 Insulin, Globin Zinc Not Applicable
5 Antiparkinson Agents Not Applicable
6 Incretins Not Applicable
7 insulin Not Applicable
8 Pharmaceutical Solutions Not Applicable
9 Glucagon-Like Peptide 1 Not Applicable
10 Narcotics
11 Cola

Interventional clinical trials:

(show all 13)
# Name Status NCT ID Phase Drugs
1 Novel Neuroimage Study in Tauopathies With Parkinsonism Active, not recruiting NCT03386669 Phase 2 F-18
2 Exploring the Relationship Between Alpha-synucleinopathy Related Non-motor Symptom and Post-op. Delirium in Elderly Patients With Spine Surgery Unknown status NCT02550626
3 Gait and REM Sleep Behavior Disorder Unknown status NCT02554331 Not Applicable
4 Biomarkers in Parkinsonian Syndromes Unknown status NCT02114242
5 North American Prodromal Synucleinopathy Consortium Recruiting NCT03623672
6 Natural History Study of Synucleinopathies Recruiting NCT01799915
7 Post-prandial Hypotension and Sleepiness in Parkinson's Disease and Other Synucleinopathies Recruiting NCT02021903 Not Applicable
8 Prodromal Markers of First-degree Relatives of Patients With Psychiatric Disorders Comorbid With RBD Recruiting NCT03595475
9 Oligomeric Alpha-synuclein in Multiple System Atrophy Recruiting NCT01485549
10 Detecting Parkinson's Disease Through Speech Analysis Active, not recruiting NCT03133611 Not Applicable
11 Progression of Prodromal Markers of α-synucleinopathy Neurodegeneration in the FDRs of Patients With RBD Not yet recruiting NCT03671772
12 Gut Microbiota Across Early Stages of Synucleinopathy: From High-risk Relatives, REM Sleep Behavior Disorder to Early Parkinson's Disease Not yet recruiting NCT03645226
13 Gut Microbiota and Parkinson's Disease Not yet recruiting NCT03710668

Search NIH Clinical Center for Synucleinopathy

Genetic Tests for Synucleinopathy

Anatomical Context for Synucleinopathy

MalaCards organs/tissues related to Synucleinopathy:

41
Brain, Eye, Olfactory Bulb, Amygdala, Temporal Lobe, Cerebellum, Cortex

Publications for Synucleinopathy

Articles related to Synucleinopathy:

(show top 50) (show all 235)
# Title Authors Year
1
Alzheimer disease biomarkers and synucleinopathy. ( 29467302 )
2018
2
Chronic Caffeine Treatment Protects Against I+-Synucleinopathy by Reestablishing Autophagy Activity in the Mouse Striatum. ( 29770111 )
2018
3
An Enriched Environment Ameliorates Oxidative Stress and Olfactory Dysfunction in Parkinson's Disease with I+-Synucleinopathy. ( 29707965 )
2018
4
Impaired Fast Network Oscillations and Mitochondrial Dysfunction in a Mouse Model of Alpha-synucleinopathy (A30P). ( 29524634 )
2018
5
Cerebrospinal Fluid Total and Phosphorylated α-Synuclein in Patients with Creutzfeldt-Jakob Disease and Synucleinopathy. ( 30136097 )
2018
6
The "when" and "where" of α-synucleinopathies: Insights from REM sleep behavior disorder. ( 30089622 )
2018
7
Impact of DHA intake in a mouse model of synucleinopathy. ( 29229294 )
2018
8
CSF tau and β-amyloid predict cerebral synucleinopathy in autopsied Lewy body disorders. ( 29467305 )
2018
9
Graphene quantum dots prevent α-synucleinopathy in Parkinson's disease. ( 29988049 )
2018
10
Region-Specific Effects of Immunotherapy With Antibodies Targeting α-synuclein in a Transgenic Model of Synucleinopathy. ( 30022929 )
2018
11
Activation of the DNA damage response in vivo in synucleinopathy models of Parkinson's disease. ( 30050065 )
2018
12
Immunotherapy targeting toll-like receptor 2 alleviates neurodegeneration in models of synucleinopathy by modulating α-synuclein transmission and neuroinflammation. ( 30092810 )
2018
13
Quality Over Quantity: Advantages of Using Alpha-Synuclein Preformed Fibril Triggered Synucleinopathy to Model Idiopathic Parkinson's Disease. ( 30233303 )
2018
14
Skin α-synuclein deposits differ in clinical variants of synucleinopathy: an in vivo study. ( 30250046 )
2018
15
Oligodendroglial A-synucleinopathy driven neuroinflammation in multiple system atrophy. ( 30444295 )
2018
16
Engineering synucleinopathy-resistant human dopaminergic neurons by CRISPR-mediated deletion of the SNCA gene. ( 30472757 )
2018
17
Investigating the neuroprotective effect of AAV-mediated β-synuclein overexpression in a transgenic model of synucleinopathy. ( 30510219 )
2018
18
Distinct α-Synuclein strains and implications for heterogeneity among α-Synucleinopathies. ( 28751258 )
2018
19
FKBP12-immunopositive inclusions in patients with α-synucleinopathies. ( 29246765 )
2018
20
When Do α-Synucleinopathies Start? An Epidemiological Timeline: A Review. ( 29459985 )
2018
21
Influence of Drug-Carrier Polymers on Alpha-Synucleinopathies: A Neglected Aspect in New Therapies Development. ( 29686999 )
2018
22
Cellular milieu imparts distinct pathological α-synuclein strains in α-synucleinopathies. ( 29743672 )
2018
23
Rapid eye movement sleep behavior disorder and the link to alpha-synucleinopathies. ( 29883833 )
2018
24
Orthostatic Hypotension as a Prodromal Marker of α-Synucleinopathies-Reply. ( 30105350 )
2018
25
Orthostatic Hypotension as a Prodromal Marker of α-Synucleinopathies. ( 30105358 )
2018
26
α-Synuclein interacts directly but reversibly with psychosine: implications for α-synucleinopathies. ( 30127535 )
2018
27
Ubiquitin specific protease-13 independently regulates parkin ubiquitination and alpha-synuclein clearance in alpha-synucleinopathies. ( 30329047 )
2018
28
LRP10 in α-synucleinopathies. ( 30507383 )
2018
29
LRP10 in α-synucleinopathies. ( 30507384 )
2018
30
LRP10 in α-synucleinopathies. ( 30507385 )
2018
31
LRP10 in α-synucleinopathies. ( 30507386 )
2018
32
LRP10 in α-synucleinopathies. ( 30507387 )
2018
33
LRP10 in α-synucleinopathies - Authors' reply. ( 30507388 )
2018
34
Predictive markers for early conversion of iRBD to neurodegenerative synucleinopathy diseases. ( 28330956 )
2017
35
Pure autonomic failure without synucleinopathy. ( 28188385 )
2017
36
Aggregation and phosphorylation of I+-synuclein with proteinase K-resistance in focal I+-synucleinopathy predominantly localized to the cardiac sympathetic nervous system. ( 28637099 )
2017
37
'Prion-like' propagation of the synucleinopathy of M83 transgenic mice depends on the mouse genotype and type of inoculum. ( 28771723 )
2017
38
Intrastriatal injection of I+-synuclein can lead to widespread synucleinopathy independent of neuroanatomic connectivity. ( 28552073 )
2017
39
Differential effects of immunotherapy with antibodies targeting I+-synuclein oligomers and fibrils in a transgenic model of synucleinopathy. ( 28476636 )
2017
40
Peripheral synucleinopathy in early Parkinson's disease: Submandibular gland needle biopsy findings. ( 28513078 )
2017
41
Glucosylceramide synthase inhibition alleviates aberrations in synucleinopathy models. ( 28223512 )
2017
42
Multiple System Atrophy: AnA Oligodendroglioneural Synucleinopathy. ( 28984582 )
2017
43
Reply to "Pure autonomic failure vs. Manifest CNS synucleinopathy: Relevance of stridor and autonomic biomarkers". ( 28472861 )
2017
44
I+-synuclein astrogliopathy: A possible specific feature in I+-synucleinopathy. ( 28244166 )
2017
45
Toll-like receptor 4 stimulation with monophosphoryl lipid A ameliorates motor deficits and nigral neurodegeneration triggered by extraneuronal I+-synucleinopathy. ( 28676095 )
2017
46
Suppression of autophagy in the brain of transgenic mice with overexpression of D953Dc-mutant I+-synuclein as an early event at synucleinopathy progression. ( 29203207 )
2017
47
The Oligomer Hypothesis in I+-Synucleinopathy. ( 28828740 )
2017
48
Pontine calcification in late stage cerebellar multiple system atrophy: a marker of synucleinopathy neurodegeneration? ( 29236831 )
2017
49
Dopamine transporter imaging deficit predicts early transition to synucleinopathy in idiopathic rapid eye movement sleep behavior disorder. ( 28833467 )
2017
50
AAV-PHP.B-Mediated Global-Scale Expression in the Mouse Nervous System Enables GBA1 Gene Therapy for Wide Protection from Synucleinopathy. ( 28882452 )
2017

Variations for Synucleinopathy

Expression for Synucleinopathy

Search GEO for disease gene expression data for Synucleinopathy.

Pathways for Synucleinopathy

Pathways related to Synucleinopathy according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.76 APP CALB1 GRM5 MAPT PRKN SNCA
2
Show member pathways
11.53 APP GRM5 SNCA
3 11.38 PRKN SNCA SNCAIP TH
4 10.76 KLK6 PRKN SNCA TH

GO Terms for Synucleinopathy

Cellular components related to Synucleinopathy according to GeneCards Suite gene sharing:

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 synapse GO:0045202 9.88 APP CALB1 CALB2 SNCA SNCB
2 perinuclear region of cytoplasm GO:0048471 9.88 APP HSF1 NEDD4 PRKN SNCA SNCG
3 synaptic vesicle GO:0008021 9.7 SNCA SNCAIP TH
4 neuron projection GO:0043005 9.7 APP CALB1 CALB2 GRM5 MAPT PRKN
5 dendritic spine GO:0043197 9.67 APP CALB1 MAPT NEDD4
6 presynapse GO:0098793 9.62 CALB2 PRKN SNCA SNCB
7 inclusion body GO:0016234 9.51 SNCA SNCB
8 axon GO:0030424 9.5 APP CALB1 MAPT PVALB SNCA SNCG
9 postsynaptic cytosol GO:0099524 9.49 CALB1 NEDD4
10 main axon GO:0044304 9.46 APP MAPT
11 neuronal cell body GO:0043025 9.23 CALB1 MAPT PVALB SNCA SNCAIP SNCG
12 cytosol GO:0005829 10.25 APP CALB1 CALB2 CHMP2B HSF1 MAPT
13 cytoplasm GO:0005737 10.19 APP CALB1 CALB2 CHMP2B GRM5 HSF1

Biological processes related to Synucleinopathy according to GeneCards Suite gene sharing:

(show all 35)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of gene expression GO:0010629 9.94 APP HSF1 MAPT PRKN
2 chemical synaptic transmission GO:0007268 9.93 GRM5 SNCA SNCB SNCG SST
3 cellular protein metabolic process GO:0044267 9.89 APP CALB1 PRKN SNCA SNCAIP
4 neuron projection development GO:0031175 9.86 APP GBA MAPT NEDD4
5 positive regulation of proteasomal ubiquitin-dependent protein catabolic process GO:0032436 9.79 GBA NUB1 PRKN
6 learning or memory GO:0007611 9.76 APP CALB1 GRM5
7 learning GO:0007612 9.76 APP GRM5 PRKN TH
8 regulation of cytosolic calcium ion concentration GO:0051480 9.75 CALB1 CALB2 PVALB
9 negative regulation of neuron death GO:1901215 9.73 GBA HSF1 PRKN SNCA
10 regulation of dopamine secretion GO:0014059 9.72 PRKN SNCA SNCG
11 adult locomotory behavior GO:0008344 9.71 APP PRKN SNCA SNCG
12 cellular response to copper ion GO:0071280 9.7 APP HSF1 SNCA
13 regulation of long-term neuronal synaptic plasticity GO:0048169 9.69 APP GRM5 SNCA
14 response to lead ion GO:0010288 9.67 APP MAPT
15 microglial cell activation GO:0001774 9.66 MAPT SNCA
16 response to metal ion GO:0010038 9.66 SNCB TH
17 neuron cellular homeostasis GO:0070050 9.65 CHMP2B PRKN
18 regulation of presynaptic cytosolic calcium ion concentration GO:0099509 9.65 CALB1 CALB2
19 supramolecular fiber organization GO:0097435 9.65 MAPT SNCA
20 synaptic transmission, dopaminergic GO:0001963 9.65 PRKN SNCA TH
21 locomotory behavior GO:0007626 9.65 APP CALB1 GRM5 PRKN TH
22 regulation of long-term synaptic potentiation GO:1900271 9.64 CALB1 CALB2
23 astrocyte activation GO:0048143 9.63 APP MAPT
24 dopamine biosynthetic process GO:0042416 9.63 SNCA TH
25 positive regulation of protein metabolic process GO:0051247 9.62 APP GBA
26 cognition GO:0050890 9.62 APP CHMP2B GRM5 TH
27 amyloid fibril formation GO:1990000 9.61 APP MAPT
28 regulation of postsynaptic cytosolic calcium ion concentration GO:0099566 9.61 CALB1 GRM5
29 mating behavior GO:0007617 9.6 APP TH
30 modulation of age-related behavioral decline GO:0090647 9.59 APP GRM5
31 dopamine uptake involved in synaptic transmission GO:0051583 9.57 PRKN SNCA
32 cellular response to manganese ion GO:0071287 9.54 APP PRKN TH
33 regulation of neurotransmitter secretion GO:0046928 9.46 PRKN SNCA SNCAIP SNCG
34 dopamine metabolic process GO:0042417 9.26 PRKN SNCA SNCAIP SNCB
35 synapse organization GO:0050808 9.1 APP GRM5 MAPT SNCA SNCB SNCG

Molecular functions related to Synucleinopathy according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.88 APP HSF1 MAPT PRKN SNCA SNCAIP
2 protein domain specific binding GO:0019904 9.56 CHMP2B NEDD4 SNCA TH
3 enzyme binding GO:0019899 9.35 APP MAPT PRKN SNCA TH
4 phospholipase binding GO:0043274 9.32 PRKN SNCA
5 cuprous ion binding GO:1903136 9.16 SNCA SNCB
6 calcium ion binding involved in regulation of presynaptic cytosolic calcium ion concentration GO:0099534 8.62 CALB1 CALB2
7 protein binding GO:0005515 10.27 APP CALB1 CHMP2B GBA GRM5 HSF1

Sources for Synucleinopathy

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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