MCID: STR001
MIFTS: 49

Striatonigral Degeneration malady

Genetic diseases, Neuronal diseases, Rare diseases categories

Summaries for Striatonigral Degeneration

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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 based summary: Striatonigral Degeneration is related to multiple system atrophy and olivopontocerebellar atrophy. An important gene associated with Striatonigral Degeneration is NUP62 (nucleoporin 62kDa), and among its related pathways are Monoamine GPCRs and Nicotine Pathway (Dopaminergic Neuron), Pharmacodynamics. 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...

Aliases & Classifications for Striatonigral Degeneration

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Sources:
10Disease Ontology, 44NINDS, 12DISEASES, 45Novoseek, 62UMLS, 57SNOMED-CT, 35MeSH, 27ICD10
See all sources

Striatonigral Degeneration, Aliases & Descriptions:

Name: Striatonigral Degeneration 10 44 12 45 62


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Genetic diseases, Rare diseases
Anatomical: Neuronal diseases


External Ids:

Disease Ontology10 DOID:4751
MeSH35 D020955
SNOMED-CT57 29618004
ICD1027 G23.2

Related Diseases for Striatonigral Degeneration

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Diseases in the Striatonigral Degeneration family:

Striatonigral Degeneration, Infantile

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

(show top 50)    (show all 67)
idRelated DiseaseScoreTop Affiliating Genes
1multiple system atrophy31.0SNCA, SLC6A3, DRD2, MAP2, RPS27A
2olivopontocerebellar atrophy30.7SNCA, MAP2
3machado-joseph disease30.5SNCA, RPS27A, SLC6A3
4dementia30.4RPS27A, SLC6A3, MAP2, SNCA
5pick disease30.3SNCA, RPS27A
6motor neuron disease30.3RPS27A, SNCA
7striatonigral degeneration, infantile10.7
8neuronitis10.5
9neuroleptic malignant syndrome10.3
10sporadic infantile bilateral striatal necrosis10.3
11saethre-chotzen syndrome10.2
12dystonia10.2
13corticobasal degeneration10.2
14multiple system atrophy, parkinsonian type10.2
15mitochondrial infantile bilateral striatal necrosis10.2
16huntington disease10.1DRD2
17neuronal intranuclear inclusion disease10.0RPS27A
18oppositional defiant disorder10.0SLC6A3
19supranuclear palsy, progressive10.0SLC6A3
20neuroaxonal dystrophy10.0RPS27A, SNCA
21vascular dementia10.0SNCA, RPS27A
22tauopathy10.0SNCA, RPS27A
23creutzfeldt-jakob disease10.0RPS27A, SNCA
24central neurocytoma10.0SNCA, MAP2
25narcolepsy10.0DRD2, DRD1
26spinocerebellar ataxia10.0SNCA, RPS27A
27ganglioglioma10.0MAP2, SNCA
28dementia, frontotemporal10.0RPS27A, SNCA
29rem sleep behavior disorder10.0SNCA, SLC6A3
30personality disorder10.0DRD2, SLC6A3
31cocaine abuse10.0SLC6A3, DRD2
32substance dependence10.0SLC6A3, DRD2
33drug dependence10.0SLC6A3, DRD2
34conduct disorder10.0DRD2, SLC6A3
35antisocial personality disorder10.0DRD2, SLC6A3
36myositis10.0RPS27A, SNCA
37alcohol dependence10.0DRD2, DRD1
38cocaine dependence10.0DRD2, SLC6A3
39essential tremor10.0SLC6A3, SNCA
40substance abuse10.0SLC6A3, DRD2
41dementia, lewy body10.0SLC6A3, SNCA
42wilson disease10.0SLC6A3, DRD2
43gilles de la tourette syndrome10.0SLC6A3, DRD2
44anxiety disorder10.0DRD2, SLC6A3
45amyloidosis10.0RPS27A, SNCA
46nicotine dependence, protection against10.0SLC6A3, DRD2
47transient cerebral ischemia10.0RPS27A, MAP2, DRD1
48pathological gambling10.0DRD2, DRD1, SLC6A3
49drug addiction10.0DRD1, SLC6A3, DRD2
50alcohol abuse10.0SLC6A3, DRD2, RPS27A

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



Diseases related to striatonigral degeneration

Symptoms for Striatonigral Degeneration

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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:

33
Brain, Medulla oblongata

Animal Models for Striatonigral Degeneration or affiliated genes

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MGI Mouse Phenotypes related to Striatonigral Degeneration:

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

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
Neuropsychological deficits accompanying striatonigral degeneration. (1955531)
1991
33
Striatonigral degeneration, olivopontocerebellar atrophy and "atypical" Pick disease. (1711756)
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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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
1
Show member pathways
9.5DRD1, DRD2
2
Show member pathways
Nicotine Activity on Dopaminergic Neurons38
9.5DRD2, DRD1
39.5DRD2, DRD1
49.2SNCA, SLC6A3
59.2SLC6A3, SNCA
6
Show member pathways
8.8NUP62, RPS27A, SLC6A3
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
8.7SNCA, RPS27A, SLC6A3
10
Show member pathways
8.3DRD2, DRD1, SLC6A3, SNCA
11
Show member pathways
7.9SLC6A3, NUP62, SNCA, DRD2, DRD1

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 38393459.9DRD2, DRD1
2eedq459.9DRD1, DRD2
3cis flupentixol309.8DRD2, DRD1
4eticlopride45 3010.8DRD2, DRD1
5thioflavine s459.8SNCA, RPS27A
6zuclopenthixol45 51 1311.7DRD1, DRD2
7(+)-butaclamol45 3010.7DRD2, DRD1
8sch 23390459.7DRD2, DRD1
9ibzm459.6SLC6A3, DRD2
101-benzyl-1,2,3,4-tetrahydroisoquinoline459.6SLC6A3, SNCA
11thiothixene30 1310.5DRD2, DRD1
12minaprine45 1310.5DRD1, DRD2
13alpha-methyl-p-tyrosine459.5DRD2, SLC6A3
14tetrabenazine45 30 61 51 1313.5DRD2, SLC6A3
15loxapine30 45 1311.4DRD1, DRD2
16flupenthixol45 51 1311.2DRD2, DRD1
17d amphetamine459.1DRD2, DRD1, SLC6A3
18raclopride45 3010.1DRD1, DRD2, SLC6A3
19methylphenidate45 51 1311.1SLC6A3, DRD2, DRD1
20silver459.1RPS27A, MAP2, SNCA
21sulpiride45 30 1311.1SLC6A3, DRD1, DRD2
22trimipramine45 51 1311.1DRD2, SLC6A3, DRD1
23pramipexole45 30 51 1312.1DRD2, SLC6A3, DRD1
24quinpirole45 3010.1DRD1, SLC6A3, DRD2
25ketamine45 30 1311.1DRD1, SLC6A3, DRD2
26spec-t459.1DRD2, DRD1, SLC6A3
27quetiapine45 51 30 26 1313.1DRD2, SLC6A3, DRD1
28bromocriptine45 30 1311.1DRD1, SLC6A3, DRD2
29risperidone45 51 30 61 26 1314.1DRD1, DRD2, SLC6A3
30olanzapine45 51 30 26 1313.1DRD1, DRD2, SLC6A3
31opiate459.0DRD2, SLC6A3, DRD1
32rotenone459.0SNCA, RPS27A, SLC6A3
33clozapine45 30 51 1311.9DRD2, DRD1, SLC6A3
34haloperidol45 51 30 3 1312.9DRD1, DRD2, SLC6A3
35morphine45 51 30 1311.8SLC6A3, DRD2, DRD1
36apomorphine30 45 1310.7DRD1, DRD2, SLC6A3, SNCA
37amphetamine45 51 1310.7DRD1, SNCA, DRD2, SLC6A3
38nicotine45 30 51 1311.5MAP2, DRD2, SLC6A3, DRD1
39adenylate458.3RPS27A, SNCA, DRD1, MAP2, DRD2
406-hydroxydopamine458.2DRD1, DRD2, RPS27A, SNCA, SLC6A3
41mptp458.2SLC6A3, DRD2, RPS27A, SNCA, DRD1
42levodopa45 139.2SLC6A3, RPS27A, SNCA, DRD1, DRD2
43acetylcholine45 51 30 26 1312.2SNCA, RPS27A, DRD2, DRD1, SLC6A3
44cocaine45 139.1SNCA, SLC6A3, DRD1, DRD2, MAP2
45norepinephrine45 26 1310.1DRD2, MAP2, SNCA, DRD1, SLC6A3
46gaba458.0MAP2, DRD2, RPS27A, SLC6A3, DRD1
47nmda45 309.0SLC6A3, DRD1, DRD2, MAP2, RPS27A
48methamphetamine45 51 139.6SLC6A3, SNCA, RPS27A, MAP2, DRD2, DRD1
49dopamine45 30 26 1310.6DRD2, MAP2, RPS27A, SNCA, DRD1, SLC6A3
50glutamate457.5DRD1, DRD2, MAP2, RPS27A, SNCA, SLC6A3

GO Terms for genes affiliated with Striatonigral Degeneration

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Cellular components related to Striatonigral Degeneration according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1axonGO:00304248.5SLC6A3, DRD2, SNCA
2plasma membraneGO:00058867.7SLC6A3, 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:00515849.9DRD1, DRD2
2cerebral cortex GABAergic interneuron migrationGO:00218539.9DRD2, DRD1
3peristalsisGO:00304329.9DRD2, DRD1
4phospholipase C-activating dopamine receptor signaling pathwayGO:00601589.9DRD1, DRD2
5regulation of long-term neuronal synaptic plasticityGO:00481699.9DRD2, SNCA
6dopamine metabolic processGO:00424179.9DRD2, DRD1
7striatum developmentGO:00217569.9DRD2, DRD1
8synaptic transmission, dopaminergicGO:00019639.9DRD1, DRD2
9positive regulation of cytosolic calcium ion concentration involved in phospholipase C-activating G-protein coupled signaling paGO:00514829.9DRD1, DRD2
10grooming behaviorGO:00076259.9DRD2, DRD1
11temperature homeostasisGO:00016599.9DRD2, DRD1
12long-term synaptic potentiationGO:00602919.8DRD1, SNCA
13response to amphetamineGO:00019759.8DRD2, DRD1
14negative regulation of epidermal growth factor receptor signaling pathwayGO:00420599.8RPS27A, NUP62
15positive regulation of release of sequestered calcium ion into cytosolGO:00512819.8DRD1, SNCA
16adult walking behaviorGO:00076289.7DRD1, DRD2
17visual learningGO:00085429.7DRD2, DRD1
18synapse assemblyGO:00074169.7DRD2, DRD1
19adenohypophysis developmentGO:00219849.6SLC6A3, DRD2
20response to iron ionGO:00100399.6SLC6A3, DRD2
21dopamine biosynthetic processGO:00424169.6SLC6A3, SNCA
22dopamine transportGO:00158729.5DRD1, SLC6A3
23response to cocaineGO:00422209.5DRD2, SLC6A3
24response to nicotineGO:00350949.5SLC6A3, DRD2
25regulation of dopamine metabolic processGO:00420539.5SLC6A3, DRD1
26behavioral response to cocaineGO:00481489.5DRD1, DRD2, SNCA
27positive regulation of multicellular organism growthGO:00400189.5DRD2, SLC6A3
28axonogenesisGO:00074099.3DRD2, MAP2
29sensory perception of smellGO:00076089.3SLC6A3, DRD2
30locomotory behaviorGO:00076269.2SLC6A3, DRD2
31negative regulation of apoptotic processGO:00430669.1SNCA, RPS27A, NUP62
32prepulse inhibitionGO:00601349.1SLC6A3, DRD1, DRD2
33response to drugGO:00424938.7DRD1, SNCA, DRD2, SLC6A3
34transmembrane transportGO:00550858.5NUP62, RPS27A, SLC6A3

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

idNameGO IDScoreTop Affiliating Genes
1protein N-terminus bindingGO:00474859.2SLC6A3, SNCA
2drug bindingGO:00081448.9DRD2, SLC6A3
3dopamine bindingGO:00352408.7DRD2, DRD1, SLC6A3
4protein bindingGO:00055157.2SLC6A3, DRD1, DRD2, MAP2, SNCA, NUP62

Products for genes affiliated with Striatonigral Degeneration

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Sources for Striatonigral Degeneration

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4CDC
15ExPASy
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet