MCID: STR001
MIFTS: 48

Striatonigral Degeneration malady

Neuronal diseases category
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Summaries for Striatonigral Degeneration

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44NINDS, 65Wikipedia, 47OMIM, 33MalaCards
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NINDS:44 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 progressive supranuclear palsy. An important gene associated with Striatonigral Degeneration is NUP62 (nucleoporin 62kDa), and among its related pathways are Hypothetical Network for Drug Addiction and Monoamine GPCRs. The compounds skf 38393 and eedq have been mentioned in the context of this disorder. Affiliated tissues include brain and medulla oblongata, and related mouse phenotypes are taste/olfaction and reproductive system.

Wikipedia:65 Multiple-system atrophy (MSA) is a degenerativeneurological disorder. MSA is associated with the... more...

Description from OMIM:47 500003,271930,609161

Aliases & Classifications for Striatonigral Degeneration

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8Disease Ontology, 44NINDS, 10DISEASES, 45Novoseek, 62UMLS, 47OMIM, 58SNOMED-CT, 35MeSH, 25ICD10
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Classifications:

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


Aliases & Descriptions:

striatonigral degeneration 8 44 10 45 62


External Ids:

Disease Ontology8 DOID:4751
SNOMED-CT58 29618004
MeSH35 D020955
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 68)
idRelated DiseaseScoreTop Affiliating Genes
1parkinson's disease31.0RPS27A, SLC6A3, DRD2, SNCA
2progressive supranuclear palsy30.9SLC6A3
3multiple system atrophy30.9SNCA, SLC6A3, DRD2, MAP2, RPS27A
4olivopontocerebellar atrophy30.8SNCA, MAP2
5dementia30.3RPS27A, SLC6A3, MAP2, SNCA
6motor neuron disease30.3RPS27A, SNCA
7alzheimer's disease30.2RPS27A, MAP2, DRD2, DRD1, SLC6A3, SNCA
8striatonigral degeneration infantile10.6
9neuronitis10.5
10sporadic infantile bilateral striatal necrosis10.4
11neuroleptic malignant syndrome10.3
12multiple system atrophy, parkinsonian type10.3
13machado-joseph disease10.2
14dystonia10.2
15corticobasal degeneration10.2
16mitochondrial infantile bilateral striatal necrosis10.2
17huntington's disease10.1DRD2
18neuronal intranuclear inclusion disease10.1RPS27A
19nicotine dependence10.1DRD2
20oppositional defiant disorder10.1SLC6A3
21neuroaxonal dystrophy10.0RPS27A, SNCA
22vascular dementia10.0SNCA, RPS27A
23tauopathy10.0SNCA, RPS27A
24central neurocytoma10.0SNCA, MAP2
25creutzfeldt-jakob disease10.0RPS27A, SNCA
26narcolepsy10.0DRD2, DRD1
27spinocerebellar ataxia10.0SNCA, RPS27A
28pick's disease10.0SNCA, RPS27A
29ganglioglioma10.0MAP2, SNCA
30frontotemporal dementia10.0RPS27A, SNCA
31rem sleep behavior disorder10.0SNCA, SLC6A3
32personality disorder10.0DRD2, SLC6A3
33cocaine abuse10.0SLC6A3, DRD2
34substance dependence10.0SLC6A3, DRD2
35drug dependence10.0SLC6A3, DRD2
36conduct disorder10.0DRD2, SLC6A3
37antisocial personality disorder10.0DRD2, SLC6A3
38myositis10.0RPS27A, SNCA
39alcohol dependence10.0DRD2, DRD1
40cocaine dependence10.0DRD2, SLC6A3
41essential tremor10.0SLC6A3, SNCA
42substance abuse10.0SLC6A3, DRD2
43lewy body dementia10.0SLC6A3, SNCA
44wilson disease10.0SLC6A3, DRD2
45gilles de la tourette syndrome10.0SLC6A3, DRD2
46anxiety disorder10.0DRD2, SLC6A3
47amyloidosis10.0RPS27A, SNCA
48transient cerebral ischemia10.0RPS27A, MAP2, DRD1
49pathological gambling10.0DRD2, DRD1, SLC6A3
50drug addiction10.0DRD1, SLC6A3, DRD2

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



Diseases related to striatonigral degeneration

Symptoms for Striatonigral Degeneration

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47OMIM
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Clinical features from OMIM:

500003,271930,609161

Drugs & Therapeutics for Striatonigral Degeneration

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42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Striatonigral Degeneration

Search NIH Clinical Center for Striatonigral Degeneration

Genetic Tests for Striatonigral Degeneration

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Anatomical Context for Striatonigral Degeneration

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33MalaCards
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MalaCards organs/tissues related to Striatonigral Degeneration:

33
Brain, Medulla oblongata

Animal Models for Striatonigral Degeneration or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Striatonigral Degeneration:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053948.9SLC6A3, DRD2, SNCA
2MP:00053898.2SLC6A3, DRD2, MAP2, SNCA
3MP:00053798.1SLC6A3, DRD2, MAP2, SNCA
4MP:00107718.0SNCA, DRD2, DRD1, SLC6A3
5MP:00053867.8SNCA, MAP2, DRD2, DRD1, SLC6A3
6MP:00036317.7SLC6A3, DRD1, DRD2, MAP2, SNCA
7MP:00053787.6SNCA, MAP2, DRD2, DRD1, SLC6A3

Publications for Striatonigral Degeneration

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52PubMed
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Articles related to Striatonigral Degeneration:

(show top 50)    (show all 76)
idTitleAuthorsYear
1
Intrastriatal injection of colchicine induces striatonigral degeneration in mice. (18564367)
2008
2
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). (15583958)
2005
3
Failure of caspase inhibition in the double-lesion rat model of striatonigral degeneration (multiple system atrophy). (15549330)
2005
4
Effects of pulsatile L-DOPA treatment in the double lesion rat model of striatonigral degeneration (multiple system atrophy). (15056471)
2004
5
Failure of neuronal protection by inhibition of glial activation in a rat model of striatonigral degeneration. (15372496)
2004
6
Neuropathological and behavioral changes induced by various treatment paradigms with MPTP and 3-nitropropionic acid in mice: towards a model of striatonigral degeneration (multiple system atrophy). (12764627)
2003
7
A 'single toxin-double lesion' rat model of striatonigral degeneration by intrastriatal 1-methyl-4-phenylpyridinium ion injection: a motor behavioural analysis. (12421620)
2002
8
Stimulation of growth-hormone release with clonidine does not distinguish individual cases of idiopathic Parkinson's disease from those with striatonigral degeneration. (12242540)
2002
9
Simultaneous intrastriatal 6-hydroxydopamine and quinolinic acid injection: a model of early-stage striatonigral degeneration. (11161601)
2001
10
Striatonigral degeneration with motor neuron disease. (10896276)
2000
11
Failure of neuroprotection by embryonic striatal grafts in a double lesion rat model of striatonigral degeneration (multiple system atrophy). (10877927)
2000
12
Autoradiographic study of striatal dopamine re-uptake sites and dopamine D1 and D2 receptors in a 6-hydroxydopamine and quinolinic acid double-lesion rat model of striatonigral degeneration (multiple system atrophy) and effects of embryonic ventral mesencephalic, striatal or co-grafts. (10658617)
2000
13
Complex motor disturbances in a sequential double lesion rat model of striatonigral degeneration (multiple system atrophy). (10924951)
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
An early description of striatonigral degeneration. (10836627)
2000
17
Neuropsychological follow up in patients with Parkinson's disease, striatonigral degeneration-type multisystem atrophy, and progressive supranuclear palsy. (10945805)
2000
18
Cortical and brain stem hyperexcitability in striatonigral degeneration. (9613764)
1998
19
Differentiation of idiopathic Parkinson's disease from striatonigral degeneration and progressive supranuclear palsy using iodine-123 meta-iodobenzylguanidine myocardial scintigraphy. (9562324)
1998
20
Increased basal ganglia iron in striatonigral degeneration: in vivo estimation with magnetic resonance. (9532280)
1998
21
Correlative studies of MR findings with neuropathology in Shy-Drager syndrome and striatonigral degeneration. (9594269)
1997
22
MRI in sporadic olivopontocerebellar atrophy and striatonigral degeneration. (9065575)
1997
23
Medial pallidotomy in late-stage Parkinson's disease and striatonigral degeneration. (9348415)
1997
24
Subregional loss of putaminal efferents to the basal ganglia output nuclei may cause parkinsonism in striatonigral degeneration. (8857740)
1996
25
"Pure" striatonigral degeneration and Parkinson's disease: a comparative clinical study. (7651445)
1995
26
Neuropsychological pattern of striatonigral degeneration: comparison with Parkinson's disease and progressive supranuclear palsy. (7876847)
1995
27
Striatal opioid receptor binding in Parkinson's disease, striatonigral degeneration and Steele-Richardson-Olszewski syndrome, A [11C]diprenorphine PET study. (7655890)
1995
28
Striatonigral degeneration: iron deposition in putamen correlates with the slit-like void signal of magnetic resonance imaging. (7533048)
1994
29
Striatal hypometabolism distinguishes striatonigral degeneration from Parkinson's disease. (8498828)
1993
30
Dopamine D1 receptors in Parkinson's disease and striatonigral degeneration: a positron emission tomography study. (8505636)
1993
31
Increased iron content in the putamen of patients with striatonigral degeneration. (1414283)
1992
32
Striatal D2 receptor status in patients with Parkinson's disease, striatonigral degeneration, and progressive supranuclear palsy, measured with 11C-raclopride and positron emission tomography. (1575457)
1992
33
Neuropsychological deficits accompanying striatonigral degeneration. (1955531)
1991
34
Striatonigral degeneration, olivopontocerebellar atrophy and "atypical" Pick disease. (1711756)
1991
35
Inhomogeneity of the putaminal lesion in striatonigral degeneration. (1975151)
1990
36
Catecholaminergic systems in the medulla oblongata in parkinsonian syndromes: a quantitative immunohistochemical study in Parkinson's disease, progressive supranuclear palsy, and striatonigral degeneration. (1978260)
1990
37
Striatonigral degeneration. A clinicopathological study. (2276046)
1990
38
Subdivisional involvement of nigrostriatal loop in idiopathic Parkinson's disease and striatonigral degeneration. (2557795)
1989
39
Decreased glucose utilization in the striatum and frontal lobe in probable striatonigral degeneration. (2789014)
1989
40
A case of late onset striatonigral degeneration. (2508411)
1989
41
Bromocriptine therapy in striatonigral degeneration. (3379440)
1988
42
Striatonigral degeneration. A case report and a review of the literature. (3061696)
1988
43
Binding experiments of muscarinic acetylcholine and dopamine receptors in human brains with emphasis on a case of striatonigral degeneration. (6819376)
1982
44
Striatonigral degeneration. (643319)
1978
45
Autosomal dominant striatonigral degeneration: a clinical, pathological, and biochemical study of a new genetic disorder. (1028273)
1976
46
Striatonigral degeneration. Response to levodopa therapy with pathological and neurochemical correlation. (4716845)
1973
47
Levodopa therapy in striatonigral degeneration. (4116273)
1972
48
Response to levodopa therapy in striatonigral degeneration. (4115837)
1972
49
Striatonigral degeneration. (4107377)
1971
50
Striatonigral degeneration. Clinical-pathological correlations and response to stereotaxic surgery. (4920208)
1970

Variations for Striatonigral Degeneration

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Expression for genes affiliated with Striatonigral Degeneration

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Striatonigral Degeneration

Search GEO for disease gene expression data for Striatonigral Degeneration.

Pathways for genes affiliated with Striatonigral Degeneration

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Sources:
50PathCards, 38NCBI BioSystems Database, 55Reactome, 51PharmGKB, 30KEGG, 60Thomson Reuters, 53QIAGEN
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Pathways related to Striatonigral Degeneration according to GeneCards/GeneDecks:

(show all 11)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5DRD2, DRD1
2
Show member pathways
9.5DRD1, DRD2
3
Show member pathways
Nicotine Activity on Dopaminergic Neurons38
9.5DRD1, DRD2
49.2SNCA, SLC6A3
59.2SLC6A3, SNCA
6
Show member pathways
8.8SLC6A3, RPS27A, NUP62
7
Show member pathways
8.7DRD2, DRD1, SLC6A3
8
Show member pathways
8.7SLC6A3, DRD1, DRD2
9
Show member pathways
Proteasome Degradation38
Immune response Antigen presentation by MHC class I60
8.7SLC6A3, RPS27A, SNCA
10
Show member pathways
8.3SNCA, DRD2, DRD1, SLC6A3
11
Show member pathways
7.9SLC6A3, DRD1, DRD2, SNCA, NUP62

Compounds for genes affiliated with Striatonigral Degeneration

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45Novoseek, 29IUPHAR, 51PharmGKB, 11DrugBank, 61Tocris Bioscience, 24HMDB, 3BitterDB
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Compounds related to Striatonigral Degeneration according to GeneCards/GeneDecks:

(show top 50)    (show all 98)
idCompoundScoreTop Affiliating Genes
1skf 38393459.9DRD2, DRD1
2eedq459.9DRD1, DRD2
3cis flupentixol299.8DRD2, DRD1
4eticlopride45 2910.8DRD2, DRD1
5thioflavine s459.8SNCA, RPS27A
6zuclopenthixol45 51 1111.7DRD1, DRD2
7(+)-butaclamol45 2910.7DRD2, DRD1
8sch 23390459.7DRD2, DRD1
9ibzm459.6SLC6A3, DRD2
101-benzyl-1,2,3,4-tetrahydroisoquinoline459.6SLC6A3, SNCA
11thiothixene29 1110.5DRD2, DRD1
12minaprine45 1110.5DRD1, DRD2
13alpha-methyl-p-tyrosine459.5DRD2, SLC6A3
14tetrabenazine45 29 61 51 1113.5DRD2, SLC6A3
15loxapine29 45 1111.4DRD1, DRD2
16flupenthixol45 51 1111.2DRD2, DRD1
17d amphetamine459.1DRD2, DRD1, SLC6A3
18raclopride45 2910.1DRD1, DRD2, SLC6A3
19methylphenidate45 51 1111.1SLC6A3, DRD2, DRD1
20silver459.1RPS27A, MAP2, SNCA
21sulpiride45 29 1111.1SLC6A3, DRD1, DRD2
22trimipramine45 51 1111.1DRD2, SLC6A3, DRD1
23pramipexole45 29 51 1112.1DRD2, SLC6A3, DRD1
24quinpirole45 2910.1DRD1, SLC6A3, DRD2
25ketamine45 29 1111.1DRD1, SLC6A3, DRD2
26spec-t459.1DRD2, DRD1, SLC6A3
27quetiapine45 51 29 24 1113.1DRD2, SLC6A3, DRD1
28bromocriptine45 29 1111.1DRD1, SLC6A3, DRD2
29risperidone45 51 29 61 24 1114.1DRD1, DRD2, SLC6A3
30olanzapine45 51 29 24 1113.1DRD1, DRD2, SLC6A3
31opiate459.0DRD2, SLC6A3, DRD1
32rotenone459.0SNCA, RPS27A, SLC6A3
33clozapine45 29 51 1111.9DRD2, DRD1, SLC6A3
34haloperidol45 51 29 3 1112.9DRD1, DRD2, SLC6A3
35morphine45 51 29 1111.8SLC6A3, DRD2, DRD1
36apomorphine29 45 1110.7DRD1, DRD2, SLC6A3, SNCA
37amphetamine45 51 1110.7DRD1, SNCA, DRD2, SLC6A3
38nicotine45 29 51 1111.5MAP2, DRD2, SLC6A3, DRD1
39adenylate458.3RPS27A, SNCA, DRD1, MAP2, DRD2
406-hydroxydopamine458.2DRD1, DRD2, RPS27A, SNCA, SLC6A3
41mptp458.2SLC6A3, DRD2, RPS27A, SNCA, DRD1
42levodopa45 119.2SLC6A3, RPS27A, SNCA, DRD1, DRD2
43acetylcholine45 51 29 24 1112.2SNCA, RPS27A, DRD2, DRD1, SLC6A3
44cocaine45 119.1SNCA, SLC6A3, DRD1, DRD2, MAP2
45norepinephrine45 24 1110.1DRD2, MAP2, SNCA, DRD1, SLC6A3
46gaba458.0MAP2, DRD2, RPS27A, SLC6A3, DRD1
47nmda45 299.0SLC6A3, DRD1, DRD2, MAP2, RPS27A
48methamphetamine45 51 119.6SLC6A3, SNCA, RPS27A, MAP2, DRD2, DRD1
49dopamine45 29 24 1110.6DRD2, MAP2, RPS27A, SNCA, DRD1, SLC6A3
50glutamate457.5DRD1, DRD2, MAP2, RPS27A, SNCA, 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:0304248.5SLC6A3, DRD2, SNCA
2plasma membraneGO:0058867.7SLC6A3, DRD1, DRD2, RPS27A, SNCA

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

(show all 34)
idNameGO IDScoreTop Affiliating Genes
1regulation of dopamine uptake involved in synaptic transmissionGO:0515849.9DRD2, DRD1
2cerebral cortex GABAergic interneuron migrationGO:0218539.9DRD1, DRD2
3phospholipase C-activating dopamine receptor signaling pathwayGO:0601589.9DRD2, DRD1
4regulation of long-term neuronal synaptic plasticityGO:0481699.9DRD2, SNCA
5peristalsisGO:0304329.9DRD2, DRD1
6dopamine metabolic processGO:0424179.9DRD1, DRD2
7striatum developmentGO:0217569.9DRD2, DRD1
8synaptic transmission, dopaminergicGO:0019639.9DRD2, DRD1
9positive regulation of cytosolic calcium ion concentration involved in phospholipase C-activating G-protein coupled signaling paGO:0514829.9DRD1, DRD2
10grooming behaviorGO:0076259.9DRD2, DRD1
11temperature homeostasisGO:0016599.9DRD1, DRD2
12long-term synaptic potentiationGO:0602919.8SNCA, DRD1
13response to amphetamineGO:0019759.8DRD1, DRD2
14negative regulation of epidermal growth factor receptor signaling pathwayGO:0420599.8NUP62, RPS27A
15positive regulation of release of sequestered calcium ion into cytosolGO:0512819.8SNCA, DRD1
16adult walking behaviorGO:0076289.7DRD1, DRD2
17visual learningGO:0085429.7DRD1, DRD2
18synapse assemblyGO:0074169.7DRD2, DRD1
19adenohypophysis developmentGO:0219849.6DRD2, SLC6A3
20response to iron ionGO:0100399.6SLC6A3, DRD2
21dopamine biosynthetic processGO:0424169.6SLC6A3, SNCA
22dopamine transportGO:0158729.5SLC6A3, DRD1
23response to cocaineGO:0422209.5DRD2, SLC6A3
24regulation of dopamine metabolic processGO:0420539.5DRD1, SLC6A3
25response to nicotineGO:0350949.5SLC6A3, DRD2
26behavioral response to cocaineGO:0481489.5DRD1, DRD2, SNCA
27positive regulation of multicellular organism growthGO:0400189.5SLC6A3, DRD2
28axonogenesisGO:0074099.3DRD2, MAP2
29sensory perception of smellGO:0076089.3SLC6A3, DRD2
30locomotory behaviorGO:0076269.2SLC6A3, DRD2
31negative regulation of apoptotic processGO:0430669.1NUP62, SNCA, RPS27A
32prepulse inhibitionGO:0601349.1DRD2, DRD1, SLC6A3
33response to drugGO:0424938.7SLC6A3, DRD1, DRD2, SNCA
34transmembrane transportGO:0550858.5SLC6A3, RPS27A, NUP62

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

idNameGO IDScoreTop Affiliating Genes
1protein N-terminus bindingGO:0474859.2SLC6A3, SNCA
2drug bindingGO:0081448.9SLC6A3, DRD2
3dopamine bindingGO:0352408.7SLC6A3, DRD1, DRD2
4protein bindingGO:0055157.2NUP62, SNCA, MAP2, DRD2, DRD1, SLC6A3

Products for genes affiliated with Striatonigral Degeneration

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Sources for Striatonigral Degeneration

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet