MCID: STR001
MIFTS: 38

Striatonigral Degeneration

Categories: Eye diseases, Genetic diseases, Mental diseases, Metabolic diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Striatonigral Degeneration

MalaCards integrated aliases for Striatonigral Degeneration:

Name: Striatonigral Degeneration 12 54 55 44 15 72 33

Classifications:



External Ids:

Disease Ontology 12 DOID:4751
MeSH 44 D020955
NCIt 50 C125695
SNOMED-CT 68 29618004
ICD10 33 G23.2
UMLS 72 C0270733

Summaries for Striatonigral Degeneration

NINDS : 54 Striatonigral degeneration is a neurological disorder caused by a disruption in the connection between two areas of the brain-the striatum and the substantia nigra. These two areas work together to enable balance and movement. Striatonigral degeneration is a type of multiple system atrophy (MSA). Symptoms of the disorder resemble some of those seen in Parkinson's disease, including rigidity, instability, impaired speech, and slow movements.

MalaCards based summary : Striatonigral Degeneration is related to multiple system atrophy 1 and supranuclear palsy, progressive, 1. An important gene associated with Striatonigral Degeneration is VAC14 (VAC14 Component Of PIKFYVE Complex), and among its related pathways/superpathways are cAMP signaling pathway and Microglia Activation During Neuroinflammation: Steady-State Microglia. Affiliated tissues include brain, cerebellum and eye, and related phenotypes are behavior/neurological and mortality/aging

Wikipedia : 75 Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by autonomic... more...

Related Diseases for Striatonigral Degeneration

Diseases in the Striatonigral Degeneration family:

Striatonigral Degeneration, Infantile

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

(show top 50) (show all 75)
# Related Disease Score Top Affiliating Genes
1 multiple system atrophy 1 32.3 SNCA SLC6A3 DRD2
2 supranuclear palsy, progressive, 1 30.9 SNCA SLC6A3
3 dystonia 30.0 VAC14 SLC6A3 DRD2
4 machado-joseph disease 30.0 SNCA SLC6A3
5 olivopontocerebellar atrophy 29.8 SNCA MAP2 ATP2B3
6 parkinson disease, late-onset 29.4 SNCA SLC6A3 DRD2 DRD1
7 striatonigral degeneration, infantile 12.9
8 striatonigral degeneration, childhood-onset 12.7
9 striatonigral degeneration, infantile, mitochondrial 12.5
10 familial infantile bilateral striatal necrosis 11.8
11 sporadic infantile bilateral striatal necrosis 11.8
12 multiple system atrophy, parkinsonian type 11.1
13 pure autonomic failure 10.3
14 tremor 10.3
15 alzheimer disease 10.2
16 aceruloplasminemia 10.2
17 neuroleptic malignant syndrome 10.2
18 dysphagia 10.2
19 posttransplant acute limbic encephalitis 10.2
20 rem sleep behavior disorder 10.2 SNCA SLC6A3
21 tic disorder 10.2 SLC6A3 DRD2
22 early-onset schizophrenia 10.2 DRD2 DRD1
23 alcohol-induced mental disorder 10.1 SLC6A3 DRD2
24 alcoholic psychosis 10.1 SLC6A3 DRD2
25 oppositional defiant disorder 10.1 SLC6A3 DRD2
26 antisocial personality disorder 10.1 SLC6A3 DRD2
27 personality disorder 10.1 SLC6A3 DRD2
28 stuttering 10.1 SLC6A3 DRD2
29 perry syndrome 10.1 SNCA SLC6A3
30 amyotrophic lateral sclerosis 1 10.0
31 dementia, lewy body 10.0
32 huntington disease 10.0
33 pick disease of brain 10.0
34 adrenoleukodystrophy 10.0
35 ataxia and polyneuropathy, adult-onset 10.0
36 aphasia 10.0
37 apraxia 10.0
38 neurogenic bladder 10.0
39 lateral sclerosis 10.0
40 motor neuron disease 10.0
41 cerebellar disease 10.0
42 agnosia 10.0
43 47,xyy 10.0
44 48,xyyy 10.0
45 adrenomyeloneuropathy 10.0
46 behavioral variant of frontotemporal dementia 10.0
47 cerebellar degeneration 10.0
48 corticobasal degeneration 10.0
49 pseudobulbar affect 10.0
50 autonomic dysfunction 10.0

Graphical network of the top 20 diseases related to Striatonigral Degeneration:



Diseases related to Striatonigral Degeneration

Symptoms & Phenotypes for Striatonigral Degeneration

MGI Mouse Phenotypes related to Striatonigral Degeneration:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.8 DRD1 DRD2 MAP2 SLC6A3 SNCA VAC14
2 mortality/aging MP:0010768 9.7 DRD1 DRD2 MAP2 NUP62 SLC6A3 SNCA
3 integument MP:0010771 9.65 DRD1 DRD2 SLC6A3 SNCA VAC14
4 nervous system MP:0003631 9.43 DRD1 DRD2 MAP2 SLC6A3 SNCA VAC14
5 taste/olfaction MP:0005394 8.8 DRD2 SLC6A3 SNCA

Drugs & Therapeutics for Striatonigral Degeneration

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase II, Randomised, Placebo-Controlled, Double-Blind, Replicated Crossover, Pilot Study on the Effect of Fipamezole on Neurogenic Orthostatic Hypotension in Patients With Multiple System Atrophy or Parkinson's Disease Unknown status NCT00758849 Phase 2 Placebo;Fipamezole

Search NIH Clinical Center for Striatonigral Degeneration

Cochrane evidence based reviews: striatonigral degeneration

Genetic Tests for Striatonigral Degeneration

Anatomical Context for Striatonigral Degeneration

MalaCards organs/tissues related to Striatonigral Degeneration:

41
Brain, Cerebellum, Eye, Spinal Cord, Skin, Globus Pallidus, Subthalamic Nucleus

Publications for Striatonigral Degeneration

Articles related to Striatonigral Degeneration:

(show top 50) (show all 252)
# Title Authors PMID Year
1
Alpha-synuclein binding to rab3a in multiple system atrophy. 9 38
15854772 2005
2
Combination of dopamine transporter and D2 receptor SPECT in the diagnostic evaluation of PD, MSA, and PSP. 9 38
11921116 2002
3
Loss of dopamine uptake sites and dopamine D2 receptors in striatonigral degeneration. 9 38
10661519 2000
4
Ubiquitin-positive inclusions in different types of multiple system atrophy: distribution and specificity. 9 38
8832750 1996
5
Immunohistochemical expression of microtubule-associated protein 5 (MAP5) in glial cells in multiple system atrophy. 9 38
1517758 1992
6
VAC14 syndrome in two siblings with retinitis pigmentosa and neurodegeneration with brain iron accumulation. 38
31387860 2019
7
L-dopa response pattern in a rat model of mild striatonigral degeneration. 38
31181111 2019
8
Patterns of dopamine transporter imaging in subtypes of multiple system atrophy. 38
29573392 2018
9
Immunohistochemical and Molecular Investigations Show Alteration in the Inflammatory Profile of Multiple System Atrophy Brain. 38
29850876 2018
10
Multiple system atrophy: Building a global community - 30years of advocacy efforts. 38
29269241 2018
11
Recent advances in neuropathology, biomarkers and therapeutic approach of multiple system atrophy. 38
28860330 2018
12
Multiple System Atrophy: An Oligodendroglioneural Synucleinopathy1. 38
28984582 2018
13
Progressive striatonigral degeneration in a transgenic mouse model of multiple system atrophy: translational implications for interventional therapies. 38
29298733 2018
14
Potential clinical utility of multiple system atrophy biomarkers. 38
29023182 2017
15
Neuropathology of childhood-onset basal ganglia degeneration caused by mutation of VAC14. 38
29296614 2017
16
Perirhinal accumulation of neuronal alpha-synuclein in a multiple system atrophy patient with dementia. 38
28419566 2017
17
Multiple system atrophy: clinicopathological characteristics in Japanese patients. 38
28496050 2017
18
Early Motor Fluctuations in a Patient with Striatonigral Degeneration. 38
28101035 2016
19
Importance of (123)I-ioflupane SPECT and Myocardial MIBG Scintigraphy to Determine the Candidate of Deep Brain Stimulation for Parkinson's Disease. 38
26794041 2016
20
Coenzyme Q10 Levels Are Decreased in the Cerebellum of Multiple-System Atrophy Patients. 38
26894433 2016
21
Failure of Neuroprotection Despite Microglial Suppression by Delayed-Start Myeloperoxidase Inhibition in a Model of Advanced Multiple System Atrophy: Clinical Implications. 38
26194617 2015
22
Neuropathology of multiple system atrophy: new thoughts about pathogenesis. 38
25297524 2014
23
A rat model of striatonigral degeneration generated by simultaneous injection of 6-hydroxydopamine into the medial forebrain bundle and quinolinic acid into the striatum. 38
25408589 2014
24
Neuropathologic changes of multiple system atrophy and diffuse Lewy body disease. 38
24963680 2014
25
An update on the cerebellar subtype of multiple system atrophy. 38
26331038 2014
26
Evaluation of a multiple system atrophy model in rats using multitracer microPET. 38
21866326 2012
27
Myeloperoxidase inhibition ameliorates multiple system atrophy-like degeneration in a transgenic mouse model. 38
22161470 2012
28
Increased neuronal α-synuclein pathology associates with its accumulation in oligodendrocytes in mice modeling α-synucleinopathies. 38
23077527 2012
29
Pregnancy in multiple system atrophy: a case report. 38
22208291 2011
30
[Multiple system atrophy - synuclein and neuronal degeneration]. 38
22277386 2011
31
Metabolic activity of the subthalamic nucleus in a primate model of L-dopa-unresponsive parkinsonism. 38
20810027 2010
32
Neurodegeneration in a transgenic mouse model of multiple system atrophy is associated with altered expression of oligodendroglial-derived neurotrophic factors. 38
20445049 2010
33
[Mechanism of neuronal degeneration of multiple system atrophy]. 38
19803404 2009
34
Intrastriatal injection of colchicine induces striatonigral degeneration in mice. 38
18564367 2008
35
Multiple system atrophy: alpha-synuclein and neuronal degeneration. 38
18018485 2007
36
Clinical-radiological correlation. Report of two cases. 38
17665026 2007
37
Cellular pathology in multiple system atrophy. 38
16961071 2006
38
Evidence for dopaminergic re-innervation by embryonic allografts in an optimized rat model of the Parkinsonian variant of multiple system atrophy. 38
16325004 2005
39
[Animal models of parkinsonism]. 38
16365620 2005
40
In vivo models of multiple system atrophy. 38
16092092 2005
41
Riluzole improves motor deficits and attenuates loss of striatal neurons in a sequential double lesion rat model of striatonigral degeneration (parkinson variant of multiple system atrophy). 38
15583958 2005
42
Alpha-synuclein transgenic mice: relevance to multiple system atrophy. 38
16092093 2005
43
Grading of neuropathology in multiple system atrophy: proposal for a novel scale. 38
16092088 2005
44
Effects of riluzole on combined MPTP + 3-nitropropionic acid-induced mild to moderate striatonigral degeneration in mice. 38
15503195 2005
45
Oxidative stress in transgenic mice with oligodendroglial alpha-synuclein overexpression replicates the characteristic neuropathology of multiple system atrophy. 38
15743798 2005
46
Failure of caspase inhibition in the double-lesion rat model of striatonigral degeneration (multiple system atrophy). 38
15549330 2005
47
The spectrum of pathological involvement of the striatonigral and olivopontocerebellar systems in multiple system atrophy: clinicopathological correlations. 38
15509623 2004
48
Morning glory sign: a particular MR finding in progressive supranuclear palsy. 38
16093629 2004
49
[Diseases other than Parkinson's disease presenting with parkinsonism]. 38
15651366 2004
50
Smothering dystonia in a patient with oromandibular dystonia. 38
15389989 2004

Variations for Striatonigral Degeneration

Expression for Striatonigral Degeneration

Search GEO for disease gene expression data for Striatonigral Degeneration.

Pathways for Striatonigral Degeneration

Pathways related to Striatonigral Degeneration according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.66 DRD2 DRD1 ATP2B3
2 10.97 DRD2 DRD1
3
Show member pathways
10.87 SLC6A3 DRD2 DRD1
4 10.76 SNCA SLC6A3
5 10.3 DRD2 DRD1

GO Terms for Striatonigral Degeneration

Cellular components related to Striatonigral Degeneration according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 neuronal cell body GO:0043025 9.65 SNCA SLC6A3 MAP2
2 axon GO:0030424 9.63 SNCA SLC6A3 DRD2
3 glutamatergic synapse GO:0098978 9.58 DRD2 DRD1 ATP2B3
4 synaptic vesicle membrane GO:0030672 9.49 SNCA DRD2
5 axon terminus GO:0043679 9.43 SNCA DRD2
6 ciliary membrane GO:0060170 9.37 DRD2 DRD1
7 GABA-ergic synapse GO:0098982 9.33 DRD2 DRD1 ATP2B3
8 non-motile cilium GO:0097730 9.32 DRD2 DRD1
9 dopaminergic synapse GO:0098691 9.26 SLC6A3 DRD2
10 integral component of presynaptic membrane GO:0099056 9.13 SLC6A3 DRD2 DRD1
11 integral component of postsynaptic membrane GO:0099055 8.8 SLC6A3 DRD2 DRD1

Biological processes related to Striatonigral Degeneration according to GeneCards Suite gene sharing:

(show all 36)
# Name GO ID Score Top Affiliating Genes
1 response to drug GO:0042493 9.76 SNCA SLC6A3 DRD2 DRD1
2 negative regulation of protein phosphorylation GO:0001933 9.67 SNCA DRD2
3 excitatory postsynaptic potential GO:0060079 9.67 SNCA DRD2
4 locomotory behavior GO:0007626 9.67 SLC6A3 DRD2 DRD1
5 protein import into nucleus GO:0006606 9.66 NUP62 DRD1
6 synapse assembly GO:0007416 9.66 DRD2 DRD1
7 regulation of synaptic vesicle exocytosis GO:2000300 9.65 DRD2 DRD1
8 visual learning GO:0008542 9.65 DRD2 DRD1
9 long-term synaptic potentiation GO:0060291 9.65 SNCA DRD1
10 response to nicotine GO:0035094 9.64 SLC6A3 DRD2
11 positive regulation of multicellular organism growth GO:0040018 9.64 SLC6A3 DRD2
12 response to amphetamine GO:0001975 9.63 DRD2 DRD1
13 feeding behavior GO:0007631 9.63 DRD2 DRD1
14 adult walking behavior GO:0007628 9.62 DRD2 DRD1
15 associative learning GO:0008306 9.62 DRD2 DRD1
16 positive regulation of release of sequestered calcium ion into cytosol GO:0051281 9.61 SNCA DRD1
17 regulation of long-term neuronal synaptic plasticity GO:0048169 9.61 SNCA DRD2
18 temperature homeostasis GO:0001659 9.6 DRD2 DRD1
19 response to iron ion GO:0010039 9.59 SLC6A3 DRD2
20 dopamine receptor signaling pathway GO:0007212 9.58 DRD2 DRD1
21 striatum development GO:0021756 9.58 DRD2 DRD1
22 grooming behavior GO:0007625 9.57 DRD2 DRD1
23 negative regulation of microtubule polymerization GO:0031115 9.56 SNCA MAP2
24 regulation of dopamine metabolic process GO:0042053 9.55 SLC6A3 DRD1
25 peristalsis GO:0030432 9.54 DRD2 DRD1
26 dopamine biosynthetic process GO:0042416 9.52 SNCA SLC6A3
27 adenohypophysis development GO:0021984 9.51 SLC6A3 DRD2
28 behavioral response to cocaine GO:0048148 9.5 SNCA DRD2 DRD1
29 phospholipase C-activating dopamine receptor signaling pathway GO:0060158 9.48 DRD2 DRD1
30 dopamine transport GO:0015872 9.43 SLC6A3 DRD1
31 synaptic transmission, dopaminergic GO:0001963 9.43 SNCA DRD2 DRD1
32 cerebral cortex GABAergic interneuron migration GO:0021853 9.4 DRD2 DRD1
33 regulation of dopamine uptake involved in synaptic transmission GO:0051584 9.37 DRD2 DRD1
34 prepulse inhibition GO:0060134 9.33 SLC6A3 DRD2 DRD1
35 dopamine metabolic process GO:0042417 9.13 SNCA DRD2 DRD1
36 response to cocaine GO:0042220 8.92 SNCA SLC6A3 DRD2 DRD1

Molecular functions related to Striatonigral Degeneration according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 drug binding GO:0008144 9.32 SLC6A3 DRD2
2 kinesin binding GO:0019894 9.26 SNCA NUP62
3 Hsp70 protein binding GO:0030544 9.16 SNCA NUP62
4 dopamine neurotransmitter receptor activity GO:0004952 8.96 DRD2 DRD1
5 dopamine binding GO:0035240 8.8 SLC6A3 DRD2 DRD1

Sources for Striatonigral Degeneration

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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