MCID: STR001
MIFTS: 48

Striatonigral Degeneration malady

Neuronal diseases category
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Summaries for Striatonigral Degeneration

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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 based summary: Striatonigral Degeneration is related to progressive supranuclear palsy and olivopontocerebellar atrophy. 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...

Descriptions from OMIM:46 609161,271930,500003

Aliases & Classifications for Striatonigral Degeneration

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Sources:
8Disease Ontology, 43NINDS, 10DISEASES, 44Novoseek, 62UMLS, 34MeSH, 46OMIM, 57SNOMED-CT, 25ICD10
See all sources

Striatonigral Degeneration, Aliases & Descriptions:

Name: Striatonigral Degeneration 8 43 10 44 62


Classifications:

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


External Ids:

Disease Ontology8 DOID:4751
MeSH34 D020955
SNOMED-CT57 29618004
ICD1025 G23.2

Related Diseases for Striatonigral Degeneration

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

(show top 50)    (show all 66)
idRelated DiseaseScoreTop Affiliating Genes
1progressive supranuclear palsy31.3SLC6A3
2olivopontocerebellar atrophy30.9SNCA, MAP2
3parkinson's disease30.7SNCA, RPS27A, DRD2, SLC6A3
4motor neuron disease30.4RPS27A, SNCA
5machado-joseph disease30.3SLC6A3, RPS27A, SNCA
6multiple system atrophy30.3SNCA, RPS27A, MAP2, DRD2, SLC6A3
7dementia29.9SLC6A3, MAP2, RPS27A, SNCA
8alzheimer's disease29.3SLC6A3, DRD1, DRD2, MAP2, RPS27A, SNCA
9striatonigral degeneration infantile10.6
10neuronitis10.5
11huntington's disease10.5DRD2
12neuronal intranuclear inclusion disease10.4RPS27A
13nicotine dependence10.4DRD2
14neuroleptic malignant syndrome10.3
15sporadic infantile bilateral striatal necrosis10.3
16oppositional defiant disorder10.3SLC6A3
17neuroaxonal dystrophy10.2SNCA, RPS27A
18tauopathy10.2RPS27A, SNCA
19vascular dementia10.2RPS27A, SNCA
20dystonia10.2
21corticobasal degeneration10.2
22multiple system atrophy, parkinsonian type10.2
23creutzfeldt-jakob disease10.2SNCA, RPS27A
24central neurocytoma10.2SNCA, MAP2
25narcolepsy10.2DRD2, DRD1
26spinocerebellar ataxia10.2SNCA, RPS27A
27pick's disease10.1RPS27A, SNCA
28ganglioglioma10.1SNCA, MAP2
29frontotemporal dementia10.1SNCA, RPS27A
30rem sleep behavior disorder10.1SNCA, SLC6A3
31personality disorder10.1DRD2, SLC6A3
32cocaine abuse10.1DRD2, SLC6A3
33substance dependence10.1SLC6A3, DRD2
34drug dependence10.1DRD2, SLC6A3
35conduct disorder10.1SLC6A3, DRD2
36antisocial personality disorder10.1SLC6A3, DRD2
37myositis10.1RPS27A, SNCA
38alcohol dependence10.1DRD2, DRD1
39cocaine dependence10.1SLC6A3, DRD2
40essential tremor10.1SNCA, SLC6A3
41substance abuse10.1DRD2, SLC6A3
42lewy body dementia10.1SLC6A3, SNCA
43wilson disease10.1DRD2, SLC6A3
44gilles de la tourette syndrome10.1DRD2, SLC6A3
45anxiety disorder10.0SLC6A3, DRD2
46amyloidosis10.0SNCA, RPS27A
47transient cerebral ischemia9.9RPS27A, MAP2, DRD1
48pathological gambling9.9DRD2, DRD1, SLC6A3
49drug addiction9.9DRD2, DRD1, SLC6A3
50alcohol abuse9.9RPS27A, DRD2, SLC6A3

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



Diseases related to striatonigral degeneration

Symptoms for Striatonigral Degeneration

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Clinical features from OMIM:

609161,271930,500003

Drugs & Therapeutics for Striatonigral Degeneration

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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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MalaCards organs/tissues related to Striatonigral Degeneration:

32
Brain, Medulla oblongata

Animal Models for Striatonigral Degeneration or affiliated genes

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MGI Mouse Phenotypes related to Striatonigral Degeneration:

36
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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Articles related to Striatonigral Degeneration:

(show top 50)    (show all 77)
idTitleAuthorsYear
1
A rat model of striatonigral degeneration generated by simultaneous injection of 6-hydroxydopamine into the medial forebrain bundle and quinolinic Acid into the striatum. (25408589)
2014
2
Intrastriatal injection of colchicine induces striatonigral degeneration in mice. (18564367)
2008
3
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
4
Failure of caspase inhibition in the double-lesion rat model of striatonigral degeneration (multiple system atrophy). (15549330)
2005
5
Effects of pulsatile L-DOPA treatment in the double lesion rat model of striatonigral degeneration (multiple system atrophy). (15056471)
2004
6
Failure of neuronal protection by inhibition of glial activation in a rat model of striatonigral degeneration. (15372496)
2004
7
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
8
A 'single toxin-double lesion' rat model of striatonigral degeneration by intrastriatal 1-methyl-4-phenylpyridinium ion injection: a motor behavioural analysis. (12421620)
2002
9
Stimulation of growth-hormone release with clonidine does not distinguish individual cases of idiopathic Parkinson's disease from those with striatonigral degeneration. (12242540)
2002
10
Simultaneous intrastriatal 6-hydroxydopamine and quinolinic acid injection: a model of early-stage striatonigral degeneration. (11161601)
2001
11
Striatonigral degeneration with motor neuron disease. (10896276)
2000
12
Failure of neuroprotection by embryonic striatal grafts in a double lesion rat model of striatonigral degeneration (multiple system atrophy). (10877927)
2000
13
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
14
Complex motor disturbances in a sequential double lesion rat model of striatonigral degeneration (multiple system atrophy). (10924951)
2000
15
Loss of dopamine uptake sites and dopamine D2 receptors in striatonigral degeneration. (10661519)
2000
16
Toward a primate model of L-dopa-unresponsive parkinsonism mimicking striatonigral degeneration. (10830420)
2000
17
An early description of striatonigral degeneration. (10836627)
2000
18
Neuropsychological follow up in patients with Parkinson's disease, striatonigral degeneration-type multisystem atrophy, and progressive supranuclear palsy. (10945805)
2000
19
Cortical and brain stem hyperexcitability in striatonigral degeneration. (9613764)
1998
20
Differentiation of idiopathic Parkinson's disease from striatonigral degeneration and progressive supranuclear palsy using iodine-123 meta-iodobenzylguanidine myocardial scintigraphy. (9562324)
1998
21
Increased basal ganglia iron in striatonigral degeneration: in vivo estimation with magnetic resonance. (9532280)
1998
22
Correlative studies of MR findings with neuropathology in Shy-Drager syndrome and striatonigral degeneration. (9594269)
1997
23
MRI in sporadic olivopontocerebellar atrophy and striatonigral degeneration. (9065575)
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
Inhomogeneity of the putaminal lesion in striatonigral degeneration. (1975151)
1990
35
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
36
Striatonigral degeneration. A clinicopathological study. (2276046)
1990
37
Subdivisional involvement of nigrostriatal loop in idiopathic Parkinson's disease and striatonigral degeneration. (2557795)
1989
38
Decreased glucose utilization in the striatum and frontal lobe in probable striatonigral degeneration. (2789014)
1989
39
A case of late onset striatonigral degeneration. (2508411)
1989
40
Bromocriptine therapy in striatonigral degeneration. (3379440)
1988
41
Striatonigral degeneration. A case report and a review of the literature. (3061696)
1988
42
Binding experiments of muscarinic acetylcholine and dopamine receptors in human brains with emphasis on a case of striatonigral degeneration. (6819376)
1982
43
Striatonigral degeneration. (643319)
1978
44
Autosomal dominant striatonigral degeneration: a clinical, pathological, and biochemical study of a new genetic disorder. (1028273)
1976
45
Striatonigral degeneration. Response to levodopa therapy with pathological and neurochemical correlation. (4716845)
1973
46
Levodopa therapy in striatonigral degeneration. (4116273)
1972
47
Levodopa therapy in striatonigral degeneration. (4113323)
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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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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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 Neurons37
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 Degradation37
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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Compounds related to Striatonigral Degeneration according to GeneCards/GeneDecks:

(show top 50)    (show all 98)
idCompoundScoreTop Affiliating Genes
1skf 38393449.9DRD2, DRD1
2eedq449.9DRD1, DRD2
3cis flupentixol289.8DRD2, DRD1
4eticlopride44 2810.8DRD2, DRD1
5thioflavine s449.8SNCA, RPS27A
6zuclopenthixol44 50 1111.7DRD1, DRD2
7(+)-butaclamol44 2810.7DRD2, DRD1
8sch 23390449.7DRD2, DRD1
9ibzm449.6SLC6A3, DRD2
101-benzyl-1,2,3,4-tetrahydroisoquinoline449.6SLC6A3, SNCA
11thiothixene28 1110.5DRD2, DRD1
12minaprine44 1110.5DRD1, DRD2
13alpha-methyl-p-tyrosine449.5DRD2, SLC6A3
14tetrabenazine44 28 61 50 1113.5DRD2, SLC6A3
15loxapine28 44 1111.4DRD1, DRD2
16flupenthixol44 50 1111.2DRD2, DRD1
17d amphetamine449.1DRD2, DRD1, SLC6A3
18raclopride44 2810.1DRD1, DRD2, SLC6A3
19methylphenidate44 50 1111.1SLC6A3, DRD2, DRD1
20silver449.1RPS27A, MAP2, SNCA
21sulpiride44 28 1111.1SLC6A3, DRD1, DRD2
22trimipramine44 50 1111.1DRD2, SLC6A3, DRD1
23pramipexole44 28 50 1112.1DRD2, SLC6A3, DRD1
24quinpirole44 2810.1DRD1, SLC6A3, DRD2
25ketamine44 28 1111.1DRD1, SLC6A3, DRD2
26spec-t449.1DRD2, DRD1, SLC6A3
27quetiapine44 50 28 24 1113.1DRD2, SLC6A3, DRD1
28bromocriptine44 28 1111.1DRD1, SLC6A3, DRD2
29risperidone44 50 28 61 24 1114.1DRD1, DRD2, SLC6A3
30olanzapine44 50 28 24 1113.1DRD1, DRD2, SLC6A3
31opiate449.0DRD2, SLC6A3, DRD1
32rotenone449.0SNCA, RPS27A, SLC6A3
33clozapine44 28 50 1111.9DRD2, DRD1, SLC6A3
34haloperidol44 50 28 2 1112.9DRD1, DRD2, SLC6A3
35morphine44 50 28 1111.8SLC6A3, DRD2, DRD1
36apomorphine28 44 1110.7DRD1, DRD2, SLC6A3, SNCA
37amphetamine44 50 1110.7DRD1, SNCA, DRD2, SLC6A3
38nicotine44 28 50 1111.5MAP2, DRD2, SLC6A3, DRD1
39adenylate448.3RPS27A, SNCA, DRD1, MAP2, DRD2
406-hydroxydopamine448.2DRD1, DRD2, RPS27A, SNCA, SLC6A3
41mptp448.2SLC6A3, DRD2, RPS27A, SNCA, DRD1
42levodopa44 119.2SLC6A3, RPS27A, SNCA, DRD1, DRD2
43acetylcholine44 50 28 24 1112.2SNCA, RPS27A, DRD2, DRD1, SLC6A3
44cocaine44 119.1SNCA, SLC6A3, DRD1, DRD2, MAP2
45norepinephrine44 24 1110.1DRD2, MAP2, SNCA, DRD1, SLC6A3
46gaba448.0MAP2, DRD2, RPS27A, SLC6A3, DRD1
47nmda44 289.0SLC6A3, DRD1, DRD2, MAP2, RPS27A
48methamphetamine44 50 119.6SLC6A3, SNCA, RPS27A, MAP2, DRD2, DRD1
49dopamine44 28 24 1110.6DRD2, MAP2, RPS27A, SNCA, DRD1, SLC6A3
50glutamate447.5DRD1, DRD2, MAP2, RPS27A, SNCA, SLC6A3

GO Terms for genes affiliated with Striatonigral Degeneration

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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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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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet