MCID: STR001
MIFTS: 41

Striatonigral Degeneration malady

Neuronal category

Summaries for Striatonigral Degeneration

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44NINDS, 64Wikipedia, 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 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, fetal brain and medulla oblongata, and related mouse phenotype taste/olfaction.

Wikipedia:64 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

Sources:
8Disease Ontology, 44NINDS, 10DISEASES, 45Novoseek, 61UMLS, 35MeSH, 47OMIM, 57SNOMED-CT, 25ICD10
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Classifications:

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


Aliases & Descriptions:

striatonigral degeneration 8 44 10 45 61


External Ids:

Disease Ontology8 DOID:4751
MeSH35 D020955
SNOMED-CT57 29618004
ICD1025 G23.2

Related Diseases for Striatonigral Degeneration

Sources:
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 61)
idRelated DiseaseScoreTop Affiliating Genes
1parkinson's disease31.1RPS27A, DRD2, SNCA, SLC6A3
2progressive supranuclear palsy30.7SLC6A3, SNCA, DRD2, RPS27A
3olivopontocerebellar atrophy30.6SNCA, MAP2
4alzheimer's disease30.4RPS27A, DRD1, DRD2, SNCA, MAP2, SLC6A3
5striatonigral degeneration infantile10.5
6n syndrome10.4
7neuroleptic malignant syndrome10.3
8basal ganglia disease10.3
9multiple system atrophy, parkinsonian type10.3
10machado-joseph disease10.1
11pick's disease10.1
12corticobasal degeneration10.1
13dementia - subcortical10.1
14sporadic infantile bilateral striatal necrosis10.1
15mitochondrial infantile bilateral striatal necrosis10.1
16restless legs syndrome10.0DRD2
17nicotine dependence10.0DRD2
18prion disease10.0MAP2
19down syndrome10.0SNCA
20neuronal intranuclear inclusion disease10.0RPS27A
21heroin dependence10.0DRD2
22drug dependence10.0SLC6A3, DRD2
23rem sleep behavior disorder10.0SNCA, SLC6A3
24cocaine abuse10.0DRD2, SLC6A3
25cocaine dependence10.0DRD2, SLC6A3
26lewy body dementia10.0SLC6A3, SNCA
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

Sources:
47OMIM
See all sources

Clinical features from OMIM:

500003,271930,609161

Drugs & Therapeutics for Striatonigral Degeneration

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Striatonigral Degeneration

Drug clinical trials:

Search ClinicalTrials for Striatonigral Degeneration

Search NIH Clinical Center for Striatonigral Degeneration

Search CenterWatch for Striatonigral Degeneration

Genetic Tests for Striatonigral Degeneration

Anatomical Context for Striatonigral Degeneration

Sources:
33MalaCards
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MalaCards organs/tissues related to Striatonigral Degeneration:

33
Brain, Fetal brain, Medulla oblongata

Animal Models for Striatonigral Degeneration or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Striatonigral Degeneration:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053949.3SLC6A3, SNCA, DRD2

Publications for Striatonigral Degeneration

Sources:
51PubMed
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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
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
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
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
Motor neuron disease with dementia combined with degeneration of striatonigral and pallidoluysian systems. (11935270)
2002
8
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
9
A novel grading scale for striatonigral degeneration (multiple system atrophy). (11956953)
2002
10
Thin section MR study of the basal ganglia in the differential diagnosis between striatonigral degeneration and Parkinson disease. (11884785)
2002
11
Patterns of protein nitration in dementia with Lewy bodies and striatonigral degeneration. (11935266)
2002
12
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
13
Striatonigral degeneration with motor neuron disease. (10896276)
2000
14
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
15
Complex motor disturbances in a sequential double lesion rat model of striatonigral degeneration (multiple system atrophy). (10924951)
2000
16
Loss of dopamine uptake sites and dopamine D2 receptors in striatonigral degeneration. (10661519)
2000
17
An early description of striatonigral degeneration. (10836627)
2000
18
Visual event-related potentials in progressive supranuclear palsy, corticobasal degeneration, striatonigral degeneration, and Parkinson's disease. (10896267)
2000
19
An early description of striatonigral degeneration. (10431772)
1999
20
Cortical and brain stem hyperexcitability in striatonigral degeneration. (9613764)
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
Urinary disturbances in striatonigral degeneration and Parkinson's disease: clinical and urodynamic aspects. (9251068)
1997
25
"Pure" striatonigral degeneration and Parkinson's disease: a comparative clinical study. (7651445)
1995
26
Dopamine D1 receptors in Parkinson's disease and striatonigral degeneration: a positron emission tomography study. (8505636)
1993
27
Striatonigral degeneration with neurofibrillary tangles. (8256593)
1993
28
Neuropsychological deficits accompanying striatonigral degeneration. (1955531)
1991
29
Striatonigral degeneration, olivopontocerebellar atrophy and "atypical" Pick disease. (1711756)
1991
30
Inhomogeneity of the putaminal lesion in striatonigral degeneration. (1975151)
1990
31
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
32
Striatonigral degeneration. A clinicopathological study. (2276046)
1990
33
Striatonigral degeneration: clinical, MRI, and pathologic correlation. (2320250)
1990
34
Met-enkephalin immunoreactivity in the basal ganglia in Parkinson's disease and striatonigral degeneration. (2192300)
1990
35
Subdivisional involvement of nigrostriatal loop in idiopathic Parkinson's disease and striatonigral degeneration. (2557795)
1989
36
Decreased glucose utilization in the striatum and frontal lobe in probable striatonigral degeneration. (2789014)
1989
37
Calcineurin immunoreactivity in striatonigral degeneration. (2544073)
1989
38
Striatonigral degeneration. A case report and a review of the literature. (3061696)
1988
39
Neuroleptic malignant syndrome in striatonigral degeneration. (3255444)
1988
40
Binding experiments of muscarinic acetylcholine and dopamine receptors in human brains with emphasis on a case of striatonigral degeneration. (6819376)
1982
41
Striatonigral degeneration combined with Alzheimer's disease. (6114611)
1981
42
Striatonigral degeneration. (643319)
1978
43
Striatonigral degeneration disputed in familial disorder. (557771)
1977
44
Autosomal dominant striatonigral degeneration: a clinical, pathological, and biochemical study of a new genetic disorder. (1028273)
1976
45
Striatonigral degeneration, Alzheimer's disease, and inflammatory changes. (4795740)
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

Genetic Variations for Striatonigral Degeneration

Expression for genes affiliated with Striatonigral Degeneration

Sources:
1BioGPS, 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

Sources:
38NCBI BioSystems Database, 54Reactome, 30KEGG
See all sources

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

Sources:
45Novoseek, 29IUPHAR, 60Tocris Bioscience, 50PharmGKB, 11DrugBank, 24HMDB
See all sources

Compounds related to Striatonigral Degeneration according to GeneCards/GeneDecks:

(show top 50)    (show all 94)
idCompoundScoreTop Affiliating Genes
11-benzyl-1,2,3,4-tetrahydroisoquinoline4510.1SNCA, SLC6A3
2ibzm4510.1SLC6A3, DRD2
3alpha-methyl-p-tyrosine4510.0SLC6A3, DRD2
4tetrabenazine45 29 60 50 1113.9SLC6A3, DRD2
5thioflavine s459.9SNCA, RPS27A
6Acetophenazine119.7DRD1, DRD2
7skf 38393459.7DRD1, DRD2
8Carphenazine119.6DRD2, DRD1
9Thiethylperazine119.6DRD2, DRD1
10eticlopride29 4510.6DRD1, DRD2
11cis flupentixol299.6DRD1, DRD2
12eedq459.6DRD1, DRD2
13rotenone459.5SLC6A3, SNCA, RPS27A
14nmda45 2910.5RPS27A, MAP2, SLC6A3
15Triflupromazine119.5DRD1, DRD2
16(+)-butaclamol29 4510.5DRD1, DRD2
17sch 23390459.4DRD1, DRD2
18zuclopenthixol45 50 1111.4DRD1, DRD2
19d amphetamine459.3DRD1, DRD2, SLC6A3
20raclopride45 2910.3SLC6A3, DRD2, DRD1
21trimipramine50 45 1111.3DRD1, DRD2, SLC6A3
22sulpiride45 29 1111.3DRD1, DRD2, SLC6A3
23Acepromazine119.3DRD2, DRD1
24methylphenidate45 50 1111.3DRD1, DRD2, SLC6A3
25pramipexole45 50 29 1112.3DRD1, DRD2, SLC6A3
26quinpirole45 2910.3SLC6A3, DRD2, DRD1
27ketamine45 29 1111.3SLC6A3, DRD2, DRD1
28spec-t459.3SLC6A3, DRD2, DRD1
29quetiapine45 50 29 11 2413.3SLC6A3, DRD2, DRD1
30risperidone29 45 50 60 11 2414.2DRD1, DRD2, SLC6A3
31bromocriptine29 45 1111.2DRD1, DRD2, SLC6A3
32thiothixene29 1110.2DRD2, DRD1
33olanzapine50 45 29 11 2413.2DRD1, DRD2, SLC6A3
34silver459.1RPS27A, SNCA, MAP2
35opiate459.1DRD1, DRD2, SLC6A3
36loxapine29 45 1111.0DRD2, DRD1
37apomorphine45 29 1111.0DRD1, DRD2, SNCA, SLC6A3
38amphetamine45 1110.0SLC6A3, SNCA, DRD2, DRD1
39nicotine45 50 29 1111.8DRD1, DRD2, MAP2, SLC6A3
406-hydroxydopamine458.5RPS27A, DRD1, DRD2, SNCA, SLC6A3
41mptp458.5SLC6A3, SNCA, DRD2, DRD1, RPS27A
42levodopa45 119.5RPS27A, DRD1, DRD2, SNCA, SLC6A3
43acetylcholine45 50 29 11 2412.5RPS27A, DRD1, DRD2, SNCA, SLC6A3
44cocaine45 119.4SLC6A3, MAP2, SNCA, DRD2, DRD1
45norepinephrine45 11 2410.4SLC6A3, MAP2, SNCA, DRD2, DRD1
46gaba458.4RPS27A, DRD1, DRD2, MAP2, SLC6A3
47adenylate458.3RPS27A, DRD1, DRD2, SNCA, MAP2
48methamphetamine45 50 1110.0SLC6A3, MAP2, SNCA, DRD2, DRD1, RPS27A
49dopamine45 29 11 2411.0RPS27A, DRD1, DRD2, SNCA, MAP2, SLC6A3
50glutamate458.0RPS27A, DRD1, DRD2, SNCA, MAP2, SLC6A3

GO Terms for genes affiliated with Striatonigral Degeneration

Sources:
16Gene Ontology
See all sources

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

3CDC
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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet