MCID: STR001
MIFTS: 38

Striatonigral Degeneration malady

Categories: Genetic diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Striatonigral Degeneration

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Aliases & Descriptions for Striatonigral Degeneration:

Name: Striatonigral Degeneration 10 46 47 12 36 65

Classifications:



External Ids:

Disease Ontology10 DOID:4751
ICD1027 G23.2
SNOMED-CT59 29618004
MeSH36 D020955
UMLS65 C0270733

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 striatonigral degeneration, infantile and multiple system atrophy. An important gene associated with Striatonigral Degeneration is NUP62 (Nucleoporin 62kDa), and among its related pathways are Hypothetical Network for Drug Addiction and Parkinsons Disease Pathway. Affiliated tissues include brain, prostate and b cells, and related mouse phenotypes are taste/olfaction and nervous system.

Wikipedia:68 Multiple system atrophy (MSA) is a degenerativeneurological disorder. MSA is associated with the... more...

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 61)
idRelated DiseaseScoreTop Affiliating Genes
1striatonigral degeneration, infantile12.5
2multiple system atrophy11.6
3familial infantile bilateral striatal necrosis11.4
4sporadic infantile bilateral striatal necrosis11.4
5mitochondrial infantile bilateral striatal necrosis11.2
6machado-joseph disease11.1
7esophagus carcinoma in situ10.3SLC6A3, SNCA
8olivopontocerebellar atrophy10.2
9neuronitis10.2
10supranuclear palsy, progressive atypical10.2SLC6A3, SNCA
11kluver-bucy syndrome10.2DRD2, SLC6A3
12prostate adenocarcinoma10.2DRD2, SLC6A3
13hallucinogen dependence10.2DRD2, SLC6A3
14chorioangioma10.2DRD2, SLC6A3
15intracranial aneurysm10.2DRD2, SLC6A3
16multiple system atrophy, parkinsonian type10.2
17specific language impairment10.2DRD2, SLC6A3
18arrhythmogenic right ventricular cardiomyopathy10.2DRD2, SLC6A3
19heroin dependence10.1DRD2, SLC6A3
20acute dacryocystitis10.1DRD2, SLC6A3
21chronic gonorrhea of cervix10.1DRD2, SLC6A3
22essential tremor10.1SLC6A3, SNCA
23adrenal cortical adenoma10.1SLC6A3, SNCA
24dementia10.1
25neuroleptic malignant syndrome10.1
26prostatic acinar adenocarcinoma10.1DRD2, SLC6A3
27viral exanthem10.1DRD2, SLC6A3
28vascular erectile tumor10.1SLC6A3, SNCA
29zebra body myopathy10.1DRD1, DRD2
30intracerebral cystic meningioma10.1DRD1, SLC6A3
31parkinson disease 110.0SLC6A3, SNCA
32drug dependence10.0DRD2, SLC6A3
33cocaine dependence10.0DRD1, DRD2
34pick disease9.9
35motor neuron disease9.9
36dystonia9.9
37corticobasal degeneration9.9
38parkinsonism-dystonia, infantile9.9DRD2, SLC6A3
39encephalomalacia9.9DRD2, SLC6A3
40atopic dermatitis9.9SLC6A3, SNCA
41hemifacial spasm9.8DRD2, SLC6A3
42mucinous cystadenocarcinoma9.8MAP2, SLC6A3
43maxillary sinus schneiderian papilloma9.8DRD2, SLC6A3, SNCA
44central nervous system hereditary degenerative disease9.8DRD2, SLC6A3, SNCA
45polycystic kidney disease 29.8DRD2, SLC6A3, SNCA
46spondylosis9.7DRD1, MAP2
47osteogenesis imperfecta, type ix9.7DRD2, SLC6A3
48mucopolysaccharidosis ih9.7DRD1, DRD2, SNCA
49kleptomania9.6DRD1, DRD2, SLC6A3
50opportunistic mycosis9.6DRD1, 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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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, Prostate, B cells, Cervix, Kidney, Lung, Breast

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:00053948.7DRD2, SLC6A3, SNCA
2MP:00036317.5DRD1, DRD2, MAP2, SLC6A3, SNCA
3MP:00053867.2DRD1, DRD2, MAP2, 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
1integral component of plasma membraneGO:00058878.6DRD1, DRD2, SLC6A3

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

(show all 27)
idNameGO IDScoreTop Affiliating Genes
1protein import into nucleusGO:00066069.8DRD1, NUP62
2peristalsisGO:00304329.8DRD1, DRD2
3negative regulation of cell migrationGO:00303369.8DRD1, DRD2
4response to nicotineGO:00350949.8DRD1, DRD2
5response to amphetamineGO:00019759.8DRD1, DRD2
6prepulse inhibitionGO:00601349.8DRD1, DRD2
7phospholipase C-activating dopamine receptor signaling pathwayGO:00601589.8DRD1, DRD2
8visual learningGO:00085429.8DRD1, DRD2
9long-term synaptic potentiationGO:00602919.8DRD1, SNCA
10dopamine receptor signaling pathwayGO:00072129.7DRD1, DRD2
11positive regulation of cytosolic calcium ion concentration involved in phospholipase C-activating G-protein coupled signaling pathwayGO:00514829.7DRD1, DRD2
12feeding behaviorGO:00076319.6DRD1, DRD2
13grooming behaviorGO:00076259.6DRD1, DRD2
14temperature homeostasisGO:00016599.6DRD1, DRD2
15response to morphineGO:00432789.5DRD1, DRD2
16response to ethanolGO:00454719.5DRD2, SLC6A3
17associative learningGO:00083069.5DRD1, DRD2
18regulation of dopamine metabolic processGO:00420539.4DRD1, SLC6A3
19negative regulation of circadian sleep/wake cycle, sleepGO:00423219.4DRD1, DRD2
20behavioral response to cocaineGO:00481489.4DRD1, DRD2
21dopamine transportGO:00158729.4DRD1, SLC6A3
22regulation of dopamine uptake involved in synaptic transmissionGO:00515849.3DRD1, DRD2
23regulation of long-term neuronal synaptic plasticityGO:00481699.3DRD1, DRD2, SNCA
24synaptic transmission, dopaminergicGO:00019639.2DRD1, DRD2, SNCA
25orbitofrontal cortex developmentGO:00217699.1DRD1, DRD2
26locomotory behaviorGO:00076268.9DRD1, DRD2, SLC6A3
27response to drugGO:00424938.5DRD1, DRD2, SLC6A3, SNCA

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