MCID: STR001
MIFTS: 32

Striatonigral Degeneration malady

Genetic diseases, Neuronal diseases, Rare diseases categories

Aliases & Classifications for Striatonigral Degeneration

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Sources:
10Disease Ontology, 46NINDS, 47Novoseek, 12DISEASES, 65UMLS, 36MeSH, 59SNOMED-CT, 27ICD10
See all sources

Aliases & Descriptions for Striatonigral Degeneration:

Name: Striatonigral Degeneration 10 46 47 12 65 36


Classifications:



External Ids:

Disease Ontology10 DOID:4751
MeSH36 D020955
SNOMED-CT59 29618004
ICD1027 G23.2

Summaries for Striatonigral Degeneration

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NINDS:46 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 striatonigral degeneration, infantile. An important gene associated with Striatonigral Degeneration is NUP62 (Nucleoporin 62kDa), and among its related pathways are Alpha-synuclein signaling and Parkinsons Disease Pathway. Affiliated tissues include brain and medulla oblongata, and related mouse phenotype taste/olfaction.

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 Suite gene sharing:

(show top 50)    (show all 53)
idRelated DiseaseScoreTop Affiliating Genes
1multiple system atrophy30.5DRD2, MAP2, SLC6A3, SNCA
2striatonigral degeneration, infantile10.7
3olivopontocerebellar atrophy10.5
4neuronitis10.5
5neuroleptic malignant syndrome10.3
6dementia10.3
7familial infantile bilateral striatal necrosis10.3
8sporadic infantile bilateral striatal necrosis10.3
9mitochondrial infantile bilateral striatal necrosis10.3
10pick disease10.2
11machado-joseph disease10.2
12motor neuron disease10.2
13dystonia10.2
14corticobasal degeneration10.2
15multiple system atrophy, parkinsonian type10.2
16tarlov cysts10.1DRD1, DRD2
17c8 deficiency, type i10.1DRD1, DRD2
18drug-induced mental disorder10.0DRD1, DRD2
19erythematosquamous dermatosis10.0SLC6A3, SNCA
20aleukemic leukemia cutis10.0DRD2, SLC6A3
21transient hypogammaglobulinemia10.0DRD1, MAP2
22trachea leiomyoma10.0DRD2, SLC6A3
23perrault syndrome10.0DRD2, SLC6A3
24progressive myoclonus epilepsy10.0MAP2, SNCA
25hodgkin's paragranuloma10.0DRD2, SLC6A3
26supranuclear palsy, progressive atypical10.0SLC6A3, SNCA
27schizoid personality disorder10.0DRD2, SLC6A3
28synpolydactyly10.0DRD2, SLC6A3
29tick infestation10.0DRD2, SLC6A3
30mental retardation, autosomal recessive 329.9SLC6A3, SNCA
31subacute delirium9.9DRD2, SLC6A3
32patau syndrome9.9DRD2, SLC6A3
33huntington disease9.9DRD1, DRD2, SNCA
34substance dependence9.9DRD2, SLC6A3
35pertussis9.9DRD2, SLC6A3
36eccrine adenocarcinoma9.9DRD2, SLC6A3
37essential tremor9.9SLC6A3, SNCA
38substance-induced psychosis9.8DRD2, SLC6A3
39aortic aneurysm9.8DRD2, SLC6A3
40carotid artery occlusion9.8DRD2, SLC6A3, SNCA
41mucinoses9.8DRD2, SLC6A3, SNCA
42dementia, lewy body9.8SLC6A3, SNCA
43spastic entropion9.8DRD1, DRD2, SLC6A3
44ptosis9.8DRD1, DRD2, SLC6A3
45obsessive-compulsive disorder9.8DRD1, DRD2, SLC6A3
46bacillary angiomatosis9.8DRD1, DRD2, SLC6A3
47coccidioidomycosis9.8MAP2, SLC6A3, SNCA
48attention deficit-hyperactivity disorder9.8DRD1, DRD2, SLC6A3
49alcohol dependence9.8DRD1, DRD2, SLC6A3
50nicotine dependence, protection against9.7DRD2, 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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Drugs & Therapeutics for Striatonigral Degeneration

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Interventional clinical trials:

idNameStatusNCT IDPhase
1Fipamezole in Neurogenic Orthostatic HypotensionNot yet recruitingNCT00758849Phase 2

Search NIH Clinical Center for Striatonigral Degeneration


Cochrane evidence based reviews: 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:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053949.0DRD2, SLC6A3, SNCA

Publications for Striatonigral Degeneration

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Articles related to Striatonigral Degeneration:

(show top 50)    (show all 78)
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
Subregional loss of putaminal efferents to the basal ganglia output nuclei may cause parkinsonism in striatonigral degeneration. (8857740)
1996
24
"Pure" striatonigral degeneration and Parkinson's disease: a comparative clinical study. (7651445)
1995
25
Neuropsychological pattern of striatonigral degeneration: comparison with Parkinson's disease and progressive supranuclear palsy. (7876847)
1995
26
Striatal opioid receptor binding in Parkinson's disease, striatonigral degeneration and Steele-Richardson-Olszewski syndrome, A [11C]diprenorphine PET study. (7655890)
1995
27
Striatonigral degeneration: iron deposition in putamen correlates with the slit-like void signal of magnetic resonance imaging. (7533048)
1994
28
Striatal hypometabolism distinguishes striatonigral degeneration from Parkinson's disease. (8498828)
1993
29
Dopamine D1 receptors in Parkinson's disease and striatonigral degeneration: a positron emission tomography study. (8505636)
1993
30
Increased iron content in the putamen of patients with striatonigral degeneration. (1414283)
1992
31
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
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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GO Terms for genes affiliated with Striatonigral Degeneration

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Cellular components related to Striatonigral Degeneration according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1rough endoplasmic reticulumGO:00057919.9MAP2, SNCA
2ciliary membraneGO:00601709.7DRD1, DRD2
3nonmotile primary ciliumGO:00315139.6DRD1, DRD2
4dendritic shaftGO:00431989.5DRD1, MAP2
5axon terminusGO:00436799.1DRD1, DRD2, SNCA
6dendriteGO:00304258.9DRD1, DRD2, MAP2
7postsynaptic densityGO:00140698.9DRD2, MAP2, STRN
8dendritic spineGO:00431978.7DRD1, DRD2, STRN
9axonGO:00304248.3DRD1, DRD2, SLC6A3, SNCA
10neuronal cell bodyGO:00430257.7DRD1, MAP2, SLC6A3, STRN

Biological processes related to Striatonigral Degeneration according to GeneCards Suite gene sharing:

(show all 40)
idNameGO IDScoreTop Affiliating Genes
1dopamine uptake involved in synaptic transmissionGO:005158310.1SLC6A3, SNCA
2adenohypophysis developmentGO:002198410.0DRD2, SLC6A3
3dendrite developmentGO:00163589.9MAP2, STRN
4regulation of dopamine secretionGO:00140599.9DRD2, SNCA
5positive regulation of multicellular organism growthGO:00400189.8DRD2, SLC6A3
6phospholipase C-activating dopamine receptor signaling pathwayGO:00601589.8DRD1, DRD2
7orbitofrontal cortex developmentGO:00217699.8DRD1, DRD2
8negative regulation of circadian sleep/wake cycle, sleepGO:00423219.8DRD1, DRD2
9peristalsisGO:00304329.8DRD1, DRD2
10dopamine biosynthetic processGO:00424169.8SLC6A3, SNCA
11temperature homeostasisGO:00016599.8DRD1, DRD2
12striatum developmentGO:00217569.7DRD1, DRD2
13protein import into nucleusGO:00066069.7DRD1, NUP62
14positive regulation of cytosolic calcium ion concentration involved in phospholipase C-activating G-protein coupled signaling pathwayGO:00514829.7DRD1, DRD2
15response to nicotineGO:00350949.7DRD1, DRD2
16positive regulation of release of sequestered calcium ion into cytosolGO:00512819.7DRD1, SNCA
17cerebral cortex GABAergic interneuron migrationGO:00218539.7DRD1, DRD2
18feeding behaviorGO:00076319.7DRD1, DRD2
19positive regulation of long-term synaptic potentiationGO:19002739.7DRD1, DRD2
20adult walking behaviorGO:00076289.6DRD1, DRD2
21regulation of dopamine metabolic processGO:00420539.6DRD1, SLC6A3
22long-term synaptic potentiationGO:00602919.6DRD1, SNCA
23associative learningGO:00083069.6DRD1, DRD2
24grooming behaviorGO:00076259.6DRD1, DRD2
25dopamine transportGO:00158729.5DRD1, SLC6A3
26synapse assemblyGO:00074169.5DRD1, DRD2
27response to amphetamineGO:00019759.5DRD1, DRD2
28dopamine receptor signaling pathwayGO:00072129.5DRD1, DRD2
29visual learningGO:00085429.5DRD1, DRD2
30negative regulation of cell proliferationGO:00082859.5DRD2, NUP62, STRN
31regulation of long-term neuronal synaptic plasticityGO:00481699.4DRD1, DRD2, SNCA
32response to morphineGO:00432789.4DRD1, DRD2
33regulation of dopamine uptake involved in synaptic transmissionGO:00515849.3DRD1, DRD2
34synaptic transmission, dopaminergicGO:00019639.1DRD1, DRD2, SNCA
35dopamine metabolic processGO:00424179.0DRD1, DRD2, SNCA
36behavioral response to cocaineGO:00481488.9DRD1, DRD2, SNCA
37response to drugGO:00424938.8DRD1, DRD2, SNCA
38prepulse inhibitionGO:00601348.6DRD1, DRD2, SLC6A3
39locomotory behaviorGO:00076268.3DRD1, DRD2, SLC6A3, STRN
40response to cocaineGO:00422207.9DRD1, DRD2, SLC6A3, SNCA

Molecular functions related to Striatonigral Degeneration according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1kinesin bindingGO:00198949.8NUP62, SNCA
2dopamine neurotransmitter receptor activityGO:00049529.1DRD1, DRD2
3drug bindingGO:00081448.9DRD1, DRD2
4dopamine bindingGO:00352408.6DRD1, DRD2, SLC6A3

Sources for Striatonigral Degeneration

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet