MCID: SYN058
MIFTS: 41

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 dementia, lewy body and multiple system atrophy 1. An important gene associated with Synucleinopathy is SNCA (Synuclein Alpha), and among its related pathways/superpathways are Neuroscience and Parkinsons Disease Pathway. The drugs Dopamine and Menthol have been mentioned in the context of this disorder. Affiliated tissues include brain, olfactory bulb and eye, and related phenotypes are behavior/neurological and nervous system

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 51)
# Related Disease Score Top Affiliating Genes
1 dementia, lewy body 31.6 APP GBA MAPT PRKN SNCA SNCAIP
2 multiple system atrophy 1 31.4 MAPT PRKN SNCA SNCB TH
3 dysautonomia 31.2 GBA SNCA SNCAIP
4 parkinson disease, late-onset 31.0 APP CALB1 GBA GRM5 KLK6 MAPT
5 pick disease of brain 30.8 APP MAPT SNCA
6 dementia 30.5 APP CHMP2B GBA MAPT PRKN SNCA
7 supranuclear palsy, progressive, 1 30.4 APP MAPT PRKN PVALB SNCA TH
8 hereditary late-onset parkinson disease 11.0 GBA SNCA
9 early-onset parkinson's disease 10.9 GBA PRKN SNCA
10 parkinson disease 10 10.9 PRKN SNCA
11 breast fibrosarcoma 10.9 CALB1 PVALB
12 leukoencephalopathy, hereditary diffuse, with spheroids 10.8 APP MAPT SNCA
13 parkinson disease 2, autosomal recessive juvenile 10.8 PRKN SNCA
14 progressive non-fluent aphasia 10.8 CHMP2B MAPT
15 niemann-pick disease, type c1 10.8 APP MAPT SNCA
16 behavioral variant of frontotemporal dementia 10.8 CHMP2B MAPT
17 neonatal hypoxic and ischemic brain injury 10.8 PVALB TH
18 toxic encephalopathy 10.8 APP SNCA TH
19 tremor 10.8 GBA MAPT PRKN SNCA
20 parkinson disease 3, autosomal dominant 10.8 MAPT PRKN SNCA SNCAIP
21 binswanger's disease 10.8 APP MAPT SST
22 familial idiopathic basal ganglia calcification 10.7 APP MAPT
23 aphasia 10.6 APP CHMP2B MAPT SNCA
24 restless legs syndrome 10.6 PRKN SNCA TH
25 frontotemporal dementia 10.6 APP CHMP2B MAPT SNCA
26 central nervous system disease 10.6 APP MAPT PRKN SNCA TH
27 nervous system disease 10.6 APP MAPT PRKN SNCA TH
28 semantic dementia 10.5 CHMP2B MAPT
29 postencephalitic parkinson disease 10.5 MAPT SNCA
30 disease of mental health 10.5 APP GRM5 MAPT PVALB SNCA
31 status epilepticus 10.5 CALB1 CALB2 PVALB SST
32 movement disease 10.5 GBA MAPT PRKN SNCA SNCG TH
33 neuronitis 10.3
34 kluver-bucy syndrome 10.2 APP MAPT
35 rem sleep behavior disorder 10.2
36 pure autonomic failure 10.0
37 aging 10.0
38 alzheimer disease 9.9
39 gaucher's disease 9.9
40 autonomic dysfunction 9.9
41 classic progressive supranuclear palsy syndrome 9.9
42 creutzfeldt-jakob disease 9.7
43 schizophrenia 9.7
44 lysosomal storage disease 9.7
45 cerebritis 9.7
46 sleep disorder 9.7
47 amyloidosis 9.7
48 depression 9.7
49 dysphagia 9.7
50 encephalopathy 9.7

Graphical network of the top 20 diseases related to Synucleinopathy:



Diseases related to Synucleinopathy

Symptoms & Phenotypes for Synucleinopathy

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 TH APP CALB1 CALB2 CHMP2B GBA
3 integument MP:0010771 9.86 APP GBA GRM5 HSF1 MAPT PRKN
4 no phenotypic analysis MP:0003012 9.61 PRKN PVALB SNCA SNCB SST TH
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 10)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dopamine Approved Not Applicable 51-61-6, 62-31-7 681
2
Menthol Approved Not Applicable 2216-51-5 16666
3 Dopamine Agents Not Applicable
4 Dopamine agonists Not Applicable
5 Antiparkinson Agents Not Applicable
6 Glucagon-Like Peptide 1 Not Applicable
7 Incretins Not Applicable
8 insulin Not Applicable
9 Insulin, Globin Zinc Not Applicable
10 Pharmaceutical Solutions Not Applicable

Interventional clinical trials:


# 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 Natural History Study of Synucleinopathies Recruiting NCT01799915
6 Post-prandial Hypotension and Sleepiness in Parkinson's Disease and Other Synucleinopathies Recruiting NCT02021903 Not Applicable
7 Detecting Parkinson's Disease Through Speech Analysis Recruiting NCT03133611 Not Applicable
8 Oligomeric Alpha-synuclein in Multiple System Atrophy Recruiting NCT01485549

Search NIH Clinical Center for Synucleinopathy

Genetic Tests for Synucleinopathy

Anatomical Context for Synucleinopathy

MalaCards organs/tissues related to Synucleinopathy:

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

Publications for Synucleinopathy

Articles related to Synucleinopathy:

(show top 50) (show all 183)
# 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
Autonomic dysfunction in Parkinson's disease and other synucleinopathies: Introduction to the series. ( 29436737 )
2018
6
Neuropathology of autonomic dysfunction in synucleinopathies. ( 29297596 )
2018
7
Impact of DHA intake in a mouse model of synucleinopathy. ( 29229294 )
2018
8
CSF tau and I^-amyloid predict cerebral synucleinopathy in autopsied Lewy body disorders. ( 29467305 )
2018
9
Predictive markers for early conversion of iRBD to neurodegenerative synucleinopathy diseases. ( 28330956 )
2017
10
Pure autonomic failure without synucleinopathy. ( 28188385 )
2017
11
Selective imaging of internalized proteopathic I+-synuclein seeds in primary neurons reveals mechanistic insight into transmission of synucleinopathies. ( 28611062 )
2017
12
Aggregation and phosphorylation of I+-synuclein with proteinase K-resistance in focal I+-synucleinopathy predominantly localized to the cardiac sympathetic nervous system. ( 28637099 )
2017
13
'Prion-like' propagation of the synucleinopathy of M83 transgenic mice depends on the mouse genotype and type of inoculum. ( 28771723 )
2017
14
Intrastriatal injection of I+-synuclein can lead to widespread synucleinopathy independent of neuroanatomic connectivity. ( 28552073 )
2017
15
Differential effects of immunotherapy with antibodies targeting I+-synuclein oligomers and fibrils in a transgenic model of synucleinopathy. ( 28476636 )
2017
16
Peripheral synucleinopathy in early Parkinson's disease: Submandibular gland needle biopsy findings. ( 28513078 )
2017
17
Glucosylceramide synthase inhibition alleviates aberrations in synucleinopathy models. ( 28223512 )
2017
18
REM Sleep Behavior Disorder in Parkinson's Disease and Other Synucleinopathies. ( 28513079 )
2017
19
Multiple System Atrophy: AnA Oligodendroglioneural Synucleinopathy. ( 28984582 )
2017
20
Reply to "Pure autonomic failure vs. Manifest CNS synucleinopathy: Relevance of stridor and autonomic biomarkers". ( 28472861 )
2017
21
I+-synuclein astrogliopathy: A possible specific feature in I+-synucleinopathy. ( 28244166 )
2017
22
Toll-like receptor 4 stimulation with monophosphoryl lipid A ameliorates motor deficits and nigral neurodegeneration triggered by extraneuronal I+-synucleinopathy. ( 28676095 )
2017
23
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
24
The Oligomer Hypothesis in I+-Synucleinopathy. ( 28828740 )
2017
25
Pontine calcification in late stage cerebellar multiple system atrophy: a marker of synucleinopathy neurodegeneration? ( 29236831 )
2017
26
Synucleinopathies: common features and hippocampal manifestations. ( 27826641 )
2017
27
Dopamine transporter imaging deficit predicts early transition to synucleinopathy in idiopathic rapid eye movement sleep behavior disorder. ( 28833467 )
2017
28
AAV-PHP.B-Mediated Global-Scale Expression in the Mouse Nervous System Enables GBA1 Gene Therapy for Wide Protection from Synucleinopathy. ( 28882452 )
2017
29
A Protein Aggregation Inhibitor, Leuco-Methylthioninium Bis(Hydromethanesulfonate), Decreases I+-Synuclein Inclusions in a Transgenic Mouse Model of Synucleinopathy. ( 29375308 )
2017
30
Animal models of I+-synucleinopathy for Parkinson disease drug development. ( 28747776 )
2017
31
Structural variants in SNCA gene and the implication to synucleinopathies. ( 28319736 )
2017
32
Imaging neuroinflammation to monitor I+-synucleinopathy. ( 28684246 )
2017
33
Anti-I+-synuclein immunotherapy reduces I+-synuclein propagation in the axon and degeneration in a combined viral vector and transgenic model of synucleinopathy. ( 28086964 )
2017
34
I+-Synucleinopathy in the Human Amygdala in Parkinson Disease: Differential Vulnerability of Somatostatin- and Parvalbumin-Expressing Neurons. ( 28859333 )
2017
35
Alpha-, beta- and gamma-synuclein quantification in cerebrospinal fluid by multiple reaction monitoring reveals increased concentrations in Alzheimer's and Creutzfeldt-Jakob disease but no alteration in synucleinopathies. ( 27507836 )
2016
36
Review: Novel treatment strategies targeting alpha-synuclein in multiple system atrophy as a model of synucleinopathy. ( 26924723 )
2016
37
Prevalence of Submandibular Gland Synucleinopathy in Parkinson's Disease, Dementia with Lewy Bodies and other Lewy Body Disorders. ( 26756744 )
2016
38
Widespread transneuronal propagation of I+-synucleinopathy triggered in olfactory bulb mimics prodromal Parkinson's disease. ( 27503075 )
2016
39
I+-Synuclein-induced lysosomal dysfunction occurs through disruptions in protein trafficking in human midbrain synucleinopathy models. ( 26839413 )
2016
40
FTY720/Fingolimod Reduces Synucleinopathy and Improves Gut Motility in A53T Mice: CONTRIBUTIONS OF PRO-BRAIN-DERIVED NEUROTROPHIC FACTOR (PRO-BDNF) AND MATURE BDNF. ( 27528608 )
2016
41
Repurposing the NRF2 Activator Dimethyl Fumarate as Therapy Against Synucleinopathy in Parkinson's Disease. ( 27009601 )
2016
42
'Under pressure': is there a link between orthostatic hypotension and cognitive impairment in I+-synucleinopathies? ( 27613160 )
2016
43
Aberrant adenosine A2A receptor signaling contributes to neurodegeneration and cognitive impairments in a mouse model of synucleinopathy. ( 27342081 )
2016
44
Peripheral Synucleinopathy in Early Parkinson's Disease: Submandibular Gland Needle Biopsy Findings. ( 26799362 )
2016
45
Interferon-I^ may ameliorate synucleinopathy. ( 26970330 )
2016
46
Infiltrating T lymphocytes reduce myeloid phagocytosis activity in synucleinopathy model. ( 27364890 )
2016
47
Transmission of I+-synucleinopathy from olfactory structures deep into the temporal lobe. ( 27363576 )
2016
48
BPOZ-2 Gene Delivery Ameliorates Alpha-Synucleinopathy in A53T Transgenic Mouse Model of Parkinson's Disease. ( 26916519 )
2016
49
Anti-aging treatments slow propagation of synucleinopathy by restoring lysosomal function. ( 27485532 )
2016
50
Reducing C-terminal truncation mitigates synucleinopathy and neurodegeneration in a transgenic model of multiple system atrophy. ( 27482103 )
2016

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 11.38 PRKN SNCA SNCAIP TH
3 10.76 KLK6 PRKN SNCA TH

GO Terms for Synucleinopathy

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

(show all 12)
# 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 neuron projection GO:0043005 9.8 APP CALB1 CALB2 GRM5 MAPT PRKN
4 axon GO:0030424 9.7 APP CALB1 MAPT PVALB SNCA SNCG
5 synaptic vesicle GO:0008021 9.67 SNCA SNCAIP TH
6 dendritic spine GO:0043197 9.67 APP CALB1 MAPT NEDD4
7 neuronal cell body GO:0043025 9.56 CALB1 MAPT PVALB SNCA SNCAIP SNCG
8 inclusion body GO:0016234 9.51 SNCA SNCB
9 main axon GO:0044304 9.43 APP MAPT
10 terminal bouton GO:0043195 9.1 APP CALB1 CALB2 PVALB SNCA TH
11 cytosol GO:0005829 10.27 APP CALB1 CALB2 CHMP2B HSF1 MAPT
12 cytoplasm GO:0005737 10.25 APP CALB1 CALB2 CHMP2B GRM5 HSF1

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

(show all 33)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of gene expression GO:0010629 9.92 APP HSF1 MAPT PRKN
2 chemical synaptic transmission GO:0007268 9.91 GRM5 SNCA SNCB SNCG SST
3 cellular protein metabolic process GO:0044267 9.88 APP CALB1 PRKN SNCA SNCAIP
4 neuron projection development GO:0031175 9.84 APP GBA MAPT NEDD4
5 positive regulation of proteasomal ubiquitin-dependent protein catabolic process GO:0032436 9.8 GBA NUB1 PRKN
6 learning or memory GO:0007611 9.76 APP CALB1 GRM5
7 cognition GO:0050890 9.73 CHMP2B GRM5 TH
8 regulation of cytosolic calcium ion concentration GO:0051480 9.72 CALB1 CALB2 PVALB
9 learning GO:0007612 9.71 APP GRM5 PRKN TH
10 regulation of dopamine secretion GO:0014059 9.67 PRKN SNCA SNCG
11 adult locomotory behavior GO:0008344 9.67 APP PRKN SNCA SNCG
12 response to lead ion GO:0010288 9.66 APP MAPT
13 response to metal ion GO:0010038 9.65 SNCB TH
14 microglial cell activation GO:0001774 9.65 MAPT SNCA
15 supramolecular fiber organization GO:0097435 9.65 MAPT SNCA
16 locomotory behavior GO:0007626 9.65 APP CALB1 GRM5 PRKN TH
17 regulation of long-term synaptic potentiation GO:1900271 9.64 CALB1 CALB2
18 positive regulation of protein metabolic process GO:0051247 9.64 APP GBA
19 dopamine biosynthetic process GO:0042416 9.63 SNCA TH
20 regulation of long-term neuronal synaptic plasticity GO:0048169 9.63 APP GRM5 SNCA
21 amyloid fibril formation GO:1990000 9.62 APP MAPT
22 negative regulation of neuron death GO:1901215 9.62 GBA HSF1 PRKN SNCA
23 astrocyte activation GO:0048143 9.61 APP MAPT
24 mating behavior GO:0007617 9.61 APP TH
25 synaptic transmission, dopaminergic GO:0001963 9.61 PRKN SNCA TH
26 modulation of age-related behavioral decline GO:0090647 9.6 APP GRM5
27 neuron cellular homeostasis GO:0070050 9.59 CHMP2B PRKN
28 cellular response to manganese ion GO:0071287 9.58 PRKN TH
29 dopamine uptake involved in synaptic transmission GO:0051583 9.57 PRKN SNCA
30 regulation of presynaptic cytosolic calcium ion concentration GO:0099509 9.52 CALB1 CALB2
31 regulation of neurotransmitter secretion GO:0046928 9.46 PRKN SNCA SNCAIP SNCG
32 dopamine metabolic process GO:0042417 9.26 PRKN SNCA SNCAIP SNCB
33 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 9.8 APP HSF1 MAPT PRKN SNCA SNCAIP
2 protein domain specific binding GO:0019904 9.46 CHMP2B NEDD4 SNCA TH
3 phospholipase binding GO:0043274 9.26 PRKN SNCA
4 enzyme binding GO:0019899 9.02 APP MAPT PRKN SNCA TH
5 cuprous ion binding GO:1903136 8.96 SNCA SNCB

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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