MCID: STR001
MIFTS: 43

Striatonigral Degeneration malady

Neuronal diseases category

Summaries for Striatonigral Degeneration

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43NINDS, 63Wikipedia, 46OMIM, 32MalaCards
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NINDS:43 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: Striatonigral Degeneration is related to parkinson's disease and multiple system atrophy. An important gene associated with Striatonigral Degeneration is NUP62 (nucleoporin 62kDa), and among its related pathways are Alpha-synuclein signaling and Parkinsons Disease Pathway. The compounds 1-benzyl-1,2,3,4-tetrahydroisoquinoline and ibzm have been mentioned in the context of this disorder. Affiliated tissues include brain and medulla oblongata, and related mouse phenotype taste/olfaction.

Wikipedia:63 Multiple-system atrophy (MSA) is a degenerativeneurological disorder. MSA is associated with the... more...

Description from OMIM:46 500003,271930,609161

Aliases & Classifications for Striatonigral Degeneration

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8Disease Ontology, 43NINDS, 10DISEASES, 44Novoseek, 60UMLS, 34MeSH, 46OMIM, 56SNOMED-CT, 25ICD10
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Classifications:

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


Aliases & Descriptions:

striatonigral degeneration 8 43 10 44 60


External Ids:

Disease Ontology8 DOID:4751
MeSH34 D020955
SNOMED-CT56 29618004
ICD1025 G23.2

Related Diseases for Striatonigral Degeneration

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17GeneCards, 18GeneDecks
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Diseases related to Striatonigral Degeneration via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 59)
idRelated DiseaseScoreTop Affiliating Genes
1parkinson's disease31.0RPS27A, DRD2, SNCA, SLC6A3
2multiple system atrophy30.9RPS27A, DRD2, STRN, SNCA, MAP2, SLC6A3
3progressive supranuclear palsy30.7SLC6A3, SNCA, DRD2, RPS27A
4olivopontocerebellar atrophy30.6SNCA, MAP2
5dementia30.4SLC6A3, MAP2, SNCA, RPS27A
6alzheimer's disease30.3RPS27A, DRD1, DRD2, SNCA, MAP2, SLC6A3
7lewy body dementia30.1SLC6A3, SNCA
8striatonigral degeneration infantile10.6
9neuronitis10.4
10sporadic infantile bilateral striatal necrosis10.4
11neuroleptic malignant syndrome10.3
12basal ganglia disease10.3
13multiple system atrophy, parkinsonian type10.3
14machado-joseph disease10.1
15motor neuron disease10.1
16mitochondrial infantile bilateral striatal necrosis10.1
17restless legs syndrome10.0DRD2
18nicotine dependence10.0DRD2
19prion disease10.0MAP2
20down syndrome10.0SNCA
21neuronal intranuclear inclusion disease10.0RPS27A
22heroin dependence10.0DRD2
23drug dependence10.0SLC6A3, DRD2
24rem sleep behavior disorder10.0SNCA, SLC6A3
25cocaine abuse10.0DRD2, SLC6A3
26cocaine dependence10.0DRD2, SLC6A3
27obesity10.0SLC6A3, DRD2
28substance abuse10.0SLC6A3, DRD2
29personality disorder10.0SLC6A3, DRD2
30antisocial personality disorder10.0DRD2, SLC6A3
31neuroaxonal dystrophy10.0RPS27A, SNCA
32central neurocytoma10.0SNCA, MAP2
33vascular dementia10.0SNCA, RPS27A
34tauopathy10.0SNCA, RPS27A
35anxiety disorder10.0SLC6A3, DRD2
36creutzfeldt-jakob syndrome10.0SNCA, RPS27A
37spinocerebellar ataxia10.0RPS27A, SNCA
38alcohol dependence10.0DRD2, DRD1
39substance dependence10.0SLC6A3, DRD2
40spinocerebellar ataxia type 310.0RPS27A, SNCA, SLC6A3
41alcohol abuse10.0SLC6A3, DRD2, RPS27A
42ganglioglioma10.0MAP2, SNCA
43gilles de la tourette syndrome10.0SLC6A3, DRD2
44movement disease10.0SLC6A3, SNCA, DRD2, RPS27A
45pathological gambling10.0DRD1, DRD2, SLC6A3
46drug addiction10.0SLC6A3, DRD2, DRD1
47memory impairment10.0SLC6A3, SNCA, DRD1
48psychotic disorder10.0SLC6A3, DRD2, DRD1
49obsessive-compulsive disorder10.0DRD1, DRD2, SLC6A3
50ischemia10.0SLC6A3, MAP2, SNCA, DRD2

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



Diseases related to striatonigral degeneration

Clinical Features for Striatonigral Degeneration

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46OMIM
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Clinical features from OMIM:

500003,271930,609161

Drugs & Therapeutics for Striatonigral Degeneration

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Striatonigral Degeneration

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Anatomical Context for Striatonigral Degeneration

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32MalaCards
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MalaCards organs/tissues related to Striatonigral Degeneration:

32
Brain, Medulla oblongata

Animal Models for Striatonigral Degeneration or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Striatonigral Degeneration:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053949.3SLC6A3, SNCA, DRD2

Publications for Striatonigral Degeneration

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50PubMed
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Articles related to Striatonigral Degeneration:

(show top 50)    (show all 78)
idTitleAuthorsYear
1
Intrastriatal injection of colchicine induces striatonigral degeneration in mice. (18564367)
2008
2
Failure of caspase inhibition in the double-lesion rat model of striatonigral degeneration (multiple system atrophy). (15549330)
2005
3
Effects of riluzole on combined MPTP + 3-nitropropionic acid-induced mild to moderate striatonigral degeneration in mice. (15503195)
2005
4
Effects of pulsatile L-DOPA treatment in the double lesion rat model of striatonigral degeneration (multiple system atrophy). (15056471)
2004
5
MPTP potentiates 3-nitropropionic acid-induced striatal damage in mice: reference to striatonigral degeneration. (14697318)
2004
6
Motor neuron disease with dementia combined with degeneration of striatonigral and pallidoluysian systems. (11935270)
2002
7
Dystonia is predictive of subsequent altered dopaminergic responsiveness in a chronic 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine+3-nitropropionic acid model of striatonigral degeneration in monkeys. (12457736)
2002
8
A novel grading scale for striatonigral degeneration (multiple system atrophy). (11956953)
2002
9
Thin section MR study of the basal ganglia in the differential diagnosis between striatonigral degeneration and Parkinson disease. (11884785)
2002
10
Patterns of protein nitration in dementia with Lewy bodies and striatonigral degeneration. (11935266)
2002
11
No functional effects of embryonic neuronal grafts on motor deficits in a 3-nitropropionic acid rat model of advanced striatonigral degeneration (multiple system atrophy). (11226695)
2001
12
FLAIR MRI of striatonigral degeneration. (11342686)
2001
13
Failure of neuroprotection by embryonic striatal grafts in a double lesion rat model of striatonigral degeneration (multiple system atrophy). (10877927)
2000
14
Loss of dopamine uptake sites and dopamine D2 receptors in striatonigral degeneration. (10661519)
2000
15
Toward a primate model of L-dopa-unresponsive parkinsonism mimicking striatonigral degeneration. (10830420)
2000
16
Visual event-related potentials in progressive supranuclear palsy, corticobasal degeneration, striatonigral degeneration, and Parkinson's disease. (10896267)
2000
17
Cortical and brain stem hyperexcitability in striatonigral degeneration. (9613764)
1998
18
Differentiation of idiopathic Parkinson's disease from striatonigral degeneration and progressive supranuclear palsy using iodine-123 meta-iodobenzylguanidine myocardial scintigraphy. (9562324)
1998
19
Correlative studies of MR findings with neuropathology in Shy-Drager syndrome and striatonigral degeneration. (9594269)
1997
20
Urinary disturbances in striatonigral degeneration and Parkinson's disease: clinical and urodynamic aspects. (9251068)
1997
21
Subregional loss of putaminal efferents to the basal ganglia output nuclei may cause parkinsonism in striatonigral degeneration. (8857740)
1996
22
Reversal of behavioural abnormalities by fetal allografts in a novel rat model of striatonigral degeneration. (8866493)
1996
23
"Pure" striatonigral degeneration and Parkinson's disease: a comparative clinical study. (7651445)
1995
24
Striatal opioid receptor binding in Parkinson's disease, striatonigral degeneration and Steele-Richardson-Olszewski syndrome, A [11C]diprenorphine PET study. (7655890)
1995
25
Striatonigral degeneration combined with olivopontocerebellar atrophy with subcortical dementia and hallucinatory state. (7550604)
1995
26
Striatonigral degeneration: iron deposition in putamen correlates with the slit-like void signal of magnetic resonance imaging. (7533048)
1994
27
Striatal hypometabolism distinguishes striatonigral degeneration from Parkinson's disease. (8498828)
1993
28
Dopamine D1 receptors in Parkinson's disease and striatonigral degeneration: a positron emission tomography study. (8505636)
1993
29
Neuropsychological deficits accompanying striatonigral degeneration. (1955531)
1991
30
Striatonigral degeneration, olivopontocerebellar atrophy and "atypical" Pick disease. (1711756)
1991
31
Striatonigral degeneration. A clinicopathological study. (2276046)
1990
32
Striatonigral degeneration: clinical, MRI, and pathologic correlation. (2320250)
1990
33
Subdivisional involvement of nigrostriatal loop in idiopathic Parkinson's disease and striatonigral degeneration. (2557795)
1989
34
Decreased glucose utilization in the striatum and frontal lobe in probable striatonigral degeneration. (2789014)
1989
35
A case of late onset striatonigral degeneration. (2508411)
1989
36
Calcineurin immunoreactivity in striatonigral degeneration. (2544073)
1989
37
Striatonigral degeneration. A case report and a review of the literature. (3061696)
1988
38
Neuroleptic malignant syndrome in striatonigral degeneration. (3255444)
1988
39
Binding experiments of muscarinic acetylcholine and dopamine receptors in human brains with emphasis on a case of striatonigral degeneration. (6819376)
1982
40
Striatonigral degeneration combined with Alzheimer's disease. (6114611)
1981
41
Striatonigral degeneration. (643319)
1978
42
Striatonigral degeneration disputed in familial disorder. (557771)
1977
43
Autosomal dominant striatonigral degeneration: a clinical, pathological, and biochemical study of a new genetic disorder. (1028273)
1976
44
Autosomal dominant striatonigral degeneration. A clinical, pathologic, and biochemical study of a new genetic disorder. (945867)
1976
45
Striatonigral degeneration. Response to levodopa therapy with pathological and neurochemical correlation. (4716845)
1973
46
Levodopa therapy in striatonigral degeneration. (4116273)
1972
47
Response to levodopa therapy in striatonigral degeneration. (4115837)
1972
48
Striatonigral degeneration response to levodopa therapy. (5038888)
1972
49
Striatonigral degeneration. (4107377)
1971
50
Striatonigral degeneration. Clinical-pathological correlations and response to stereotaxic surgery. (4920208)
1970

Genetic Variations for Striatonigral Degeneration

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Expression for genes affiliated with Striatonigral Degeneration

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Striatonigral Degeneration

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Pathways for genes affiliated with Striatonigral Degeneration

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37NCBI BioSystems Database, 53Reactome, 29KEGG
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Pathways related to Striatonigral Degeneration according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.7SLC6A3, SNCA
29.7SLC6A3, SNCA
39.3DRD2, DRD1
49.3DRD2, DRD1
58.9DRD1, DRD2, SLC6A3
6
Hide members
8.9SLC6A3, DRD2, DRD1

Compounds for genes affiliated with Striatonigral Degeneration

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44Novoseek, 28IUPHAR, 59Tocris Bioscience, 49PharmGKB, 11DrugBank, 24HMDB
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Compounds related to Striatonigral Degeneration according to GeneCards/GeneDecks:

(show top 50)    (show all 94)
idCompoundScoreTop Affiliating Genes
11-benzyl-1,2,3,4-tetrahydroisoquinoline4410.1SNCA, SLC6A3
2ibzm4410.1SLC6A3, DRD2
3alpha-methyl-p-tyrosine4410.0SLC6A3, DRD2
4tetrabenazine44 28 59 49 1113.9SLC6A3, DRD2
5thioflavine s449.9SNCA, RPS27A
6Acetophenazine119.7DRD1, DRD2
7skf 38393449.7DRD1, DRD2
8Carphenazine119.6DRD2, DRD1
9Thiethylperazine119.6DRD2, DRD1
10eticlopride28 4410.6DRD1, DRD2
11cis flupentixol289.6DRD1, DRD2
12eedq449.6DRD1, DRD2
13rotenone449.5SLC6A3, SNCA, RPS27A
14nmda44 2810.5RPS27A, MAP2, SLC6A3
15Triflupromazine119.5DRD1, DRD2
16(+)-butaclamol28 4410.5DRD1, DRD2
17sch 23390449.4DRD1, DRD2
18zuclopenthixol44 49 1111.4DRD1, DRD2
19d amphetamine449.3DRD1, DRD2, SLC6A3
20raclopride44 2810.3SLC6A3, DRD2, DRD1
21trimipramine49 44 1111.3DRD1, DRD2, SLC6A3
22sulpiride44 28 1111.3DRD1, DRD2, SLC6A3
23Thioproperazine119.3DRD2, DRD1
24methylphenidate44 49 1111.3DRD1, DRD2, SLC6A3
25pramipexole44 49 28 1112.3DRD1, DRD2, SLC6A3
26quinpirole44 2810.3SLC6A3, DRD2, DRD1
27ketamine44 28 1111.3SLC6A3, DRD2, DRD1
28spec-t449.3SLC6A3, DRD2, DRD1
29quetiapine44 49 28 11 2413.3SLC6A3, DRD2, DRD1
30risperidone28 44 49 59 11 2414.2DRD1, DRD2, SLC6A3
31bromocriptine28 44 1111.2DRD1, DRD2, SLC6A3
32thiothixene28 1110.2DRD2, DRD1
33olanzapine49 44 28 11 2413.2DRD1, DRD2, SLC6A3
34silver449.1RPS27A, SNCA, MAP2
35opiate449.1DRD1, DRD2, SLC6A3
36Acepromazine119.0DRD2, DRD1
37apomorphine44 28 1111.0DRD1, DRD2, SNCA, SLC6A3
38amphetamine44 1110.0SLC6A3, SNCA, DRD2, DRD1
39nicotine44 49 28 1111.8DRD1, DRD2, MAP2, SLC6A3
406-hydroxydopamine448.5RPS27A, DRD1, DRD2, SNCA, SLC6A3
41mptp448.5SLC6A3, SNCA, DRD2, DRD1, RPS27A
42levodopa44 119.5RPS27A, DRD1, DRD2, SNCA, SLC6A3
43acetylcholine44 49 28 11 2412.5RPS27A, DRD1, DRD2, SNCA, SLC6A3
44cocaine44 119.4SLC6A3, MAP2, SNCA, DRD2, DRD1
45norepinephrine44 11 2410.4SLC6A3, MAP2, SNCA, DRD2, DRD1
46gaba448.4RPS27A, DRD1, DRD2, MAP2, SLC6A3
47adenylate448.3RPS27A, DRD1, DRD2, SNCA, MAP2
48methamphetamine44 49 1110.0SLC6A3, MAP2, SNCA, DRD2, DRD1, RPS27A
49dopamine44 28 11 2411.0RPS27A, DRD1, DRD2, SNCA, MAP2, SLC6A3
50glutamate448.0RPS27A, DRD1, DRD2, SNCA, MAP2, SLC6A3

GO Terms for genes affiliated with Striatonigral Degeneration

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16Gene Ontology
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Cellular components related to Striatonigral Degeneration according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1axonGO:0304249.0SLC6A3, SNCA, DRD2
2neuronal cell bodyGO:0430259.0SLC6A3, MAP2, STRN

Biological processes related to Striatonigral Degeneration according to GeneCards/GeneDecks:

(show all 30)
idNameGO IDScoreTop Affiliating Genes
1adenohypophysis developmentGO:02198410.1SLC6A3, DRD2
2regulation of dopamine secretionGO:01405910.1DRD2, SNCA
3dopamine biosynthetic processGO:04241610.0SLC6A3, SNCA
4response to iron ionGO:01003910.0SLC6A3, DRD2
5regulation of long-term neuronal synaptic plasticityGO:04816910.0SNCA, DRD2
6response to cocaineGO:0422209.9SLC6A3, DRD2
7response to nicotineGO:0350949.9SLC6A3, DRD2
8positive regulation of multicellular organism growthGO:0400189.8DRD2, SLC6A3
9regulation of dopamine uptake involved in synaptic transmissionGO:0515849.7DRD1, DRD2
10cerebral cortex GABAergic interneuron migrationGO:0218539.7DRD2, DRD1
11phospholipase C-activating dopamine receptor signaling pathwayGO:0601589.7DRD1, DRD2
12peristalsisGO:0304329.6DRD1, DRD2
13dopamine metabolic processGO:0424179.6DRD1, DRD2
14dopamine transportGO:0158729.6SLC6A3, DRD1
15synaptic transmission, dopaminergicGO:0019639.6DRD2, DRD1
16negative regulation of epidermal growth factor receptor signaling pathwayGO:0420599.6NUP62, RPS27A
17elevation of cytosolic calcium ion concentration involved in phospholipase C-activating G-protein coupled signaling pathwayGO:0514829.6DRD1, DRD2
18regulation of dopamine metabolic processGO:0420539.6DRD1, SLC6A3
19grooming behaviorGO:0076259.6DRD1, DRD2
20locomotory behaviorGO:0076269.6DRD2, STRN, SLC6A3
21temperature homeostasisGO:0016599.6DRD2, DRD1
22positive regulation of release of sequestered calcium ion into cytosolGO:0512819.5SNCA, DRD1
23long-term synaptic potentiationGO:0602919.5DRD1, SNCA
24response to amphetamineGO:0019759.4DRD2, DRD1
25behavioral response to cocaineGO:0481489.3SNCA, DRD2, DRD1
26adult walking behaviorGO:0076289.3DRD2, DRD1
27visual learningGO:0085429.3DRD2, DRD1
28prepulse inhibitionGO:0601349.3SLC6A3, DRD2, DRD1
29synapse assemblyGO:0074169.0DRD2, DRD1
30response to drugGO:0424938.9SLC6A3, SNCA, DRD2, DRD1

Molecular functions related to Striatonigral Degeneration according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1dopamine bindingGO:0352408.9SLC6A3, DRD2, DRD1

Products for genes affiliated with Striatonigral Degeneration

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Sources for Striatonigral Degeneration

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet