MCID: STR001
MIFTS: 33

Striatonigral Degeneration

Categories: Eye diseases, Genetic diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Striatonigral Degeneration

MalaCards integrated aliases for Striatonigral Degeneration:

Name: Striatonigral Degeneration 12 54 55 44 15 73

Classifications:



External Ids:

Disease Ontology 12 DOID:4751
ICD10 33 G23.2
MeSH 44 D020955
NCIt 50 C125695
SNOMED-CT 68 29618004
UMLS 73 C0270733

Summaries for Striatonigral Degeneration

NINDS : 54 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 1. An important gene associated with Striatonigral Degeneration is VAC14 (Vac14, PIKFYVE Complex Component), and among its related pathways/superpathways are Parkinsons Disease Pathway and Alpha-synuclein signaling. Affiliated tissues include brain, cerebellum and eye, and related phenotype is taste/olfaction.

Wikipedia : 76 Multiple system atrophy (MSA), also known as Shy´┐Ż??Drager syndrome, is a rare neurodegenerative... more...

Related Diseases for Striatonigral Degeneration

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 all 43)
# Related Disease Score Top Affiliating Genes
1 striatonigral degeneration, infantile 34.1 MT-CO3 NUP62
2 multiple system atrophy 1 30.7 DRD2 SLC6A3 SNCA
3 supranuclear palsy, progressive, 1 30.5 DRD2 SLC6A3 SNCA
4 olivopontocerebellar atrophy 30.1 ATP2B3 MAP2 SNCA
5 dystonia 29.9 DRD2 SLC6A3 VAC14
6 parkinson disease, late-onset 29.4 DRD2 MAP2 SLC6A3 SNCA
7 striatonigral degeneration, childhood-onset 12.5
8 striatonigral degeneration, infantile, mitochondrial 12.3
9 familial infantile bilateral striatal necrosis 11.6
10 sporadic infantile bilateral striatal necrosis 11.6
11 multiple system atrophy, parkinsonian type 10.4
12 alzheimer disease 10.2
13 dementia 10.2
14 neuroleptic malignant syndrome 10.2
15 motor neuron disease 10.1
16 corticobasal degeneration 10.1
17 dementia - subcortical 10.1
18 rem sleep behavior disorder 10.1 SLC6A3 SNCA
19 alcohol-induced mental disorder 10.0 DRD2 SLC6A3
20 alcoholic psychosis 10.0 DRD2 SLC6A3
21 pathological gambling 10.0 DRD2 SLC6A3
22 perry syndrome 10.0 SLC6A3 SNCA
23 oppositional defiant disorder 10.0 DRD2 SLC6A3
24 tic disorder 10.0 DRD2 SLC6A3
25 antisocial personality disorder 10.0 DRD2 SLC6A3
26 personality disorder 10.0 DRD2 SLC6A3
27 machado-joseph disease 10.0 SLC6A3 SNCA
28 stuttering 10.0 DRD2 SLC6A3
29 cocaine dependence 10.0 DRD2 SLC6A3
30 essential tremor 10.0 SLC6A3 SNCA
31 conduct disorder 10.0 DRD2 SLC6A3
32 substance abuse 10.0 DRD2 SLC6A3
33 eating disorder 10.0 DRD2 SLC6A3
34 synucleinopathy 10.0 SLC6A3 SNCA
35 migraine with aura 9.9 DRD2 SLC6A3
36 substance dependence 9.9 DRD2 SLC6A3
37 dementia, lewy body 9.9 SLC6A3 SNCA
38 cocaine abuse 9.9 DRD2 SLC6A3 SNCA
39 movement disease 9.9 DRD2 SLC6A3 SNCA
40 disease of mental health 9.9 DRD2 SLC6A3 SNCA
41 restless legs syndrome 9.9 DRD2 SLC6A3 SNCA
42 tobacco addiction 9.8 DRD2 SLC6A3
43 toxic encephalopathy 9.8 MAP2 SLC6A3 SNCA

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



Diseases related to Striatonigral Degeneration

Symptoms & Phenotypes for Striatonigral Degeneration

MGI Mouse Phenotypes related to Striatonigral Degeneration:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 taste/olfaction MP:0005394 8.8 DRD2 SLC6A3 SNCA

Drugs & Therapeutics for Striatonigral Degeneration

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Fipamezole in Neurogenic Orthostatic Hypotension Unknown status NCT00758849 Phase 2 Placebo;Fipamezole

Search NIH Clinical Center for Striatonigral Degeneration

Cochrane evidence based reviews: striatonigral degeneration

Genetic Tests for Striatonigral Degeneration

Anatomical Context for Striatonigral Degeneration

MalaCards organs/tissues related to Striatonigral Degeneration:

41
Brain, Cerebellum, Eye, Medulla Oblongata

Publications for Striatonigral Degeneration

Articles related to Striatonigral Degeneration:

(show top 50) (show all 82)
# Title Authors Year
1
Early Motor Fluctuations in a Patient with Striatonigral Degeneration. ( 28101035 )
2016
2
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
3
Intrastriatal injection of colchicine induces striatonigral degeneration in mice. ( 18564367 )
2008
4
Effects of riluzole on combined MPTP + 3-nitropropionic acid-induced mild to moderate striatonigral degeneration in mice. ( 15503195 )
2005
5
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
6
Failure of caspase inhibition in the double-lesion rat model of striatonigral degeneration (multiple system atrophy). ( 15549330 )
2005
7
MPTP potentiates 3-nitropropionic acid-induced striatal damage in mice: reference to striatonigral degeneration. ( 14697318 )
2004
8
Failure of neuronal protection by inhibition of glial activation in a rat model of striatonigral degeneration. ( 15372496 )
2004
9
Effects of pulsatile L-DOPA treatment in the double lesion rat model of striatonigral degeneration (multiple system atrophy). ( 15056471 )
2004
10
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
11
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
12
Patterns of protein nitration in dementia with Lewy bodies and striatonigral degeneration. ( 11935266 )
2002
13
Thin section MR study of the basal ganglia in the differential diagnosis between striatonigral degeneration and Parkinson disease. ( 11884785 )
2002
14
A 'single toxin-double lesion' rat model of striatonigral degeneration by intrastriatal 1-methyl-4-phenylpyridinium ion injection: a motor behavioural analysis. ( 12421620 )
2002
15
Stimulation of growth-hormone release with clonidine does not distinguish individual cases of idiopathic Parkinson's disease from those with striatonigral degeneration. ( 12242540 )
2002
16
A novel grading scale for striatonigral degeneration (multiple system atrophy). ( 11956953 )
2002
17
Simultaneous intrastriatal 6-hydroxydopamine and quinolinic acid injection: a model of early-stage striatonigral degeneration. ( 11161601 )
2001
18
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
19
FLAIR MRI of striatonigral degeneration. ( 11342686 )
2001
20
Visual event-related potentials in progressive supranuclear palsy, corticobasal degeneration, striatonigral degeneration, and Parkinson's disease. ( 10896267 )
2000
21
Toward a primate model of L-dopa-unresponsive parkinsonism mimicking striatonigral degeneration. ( 10830420 )
2000
22
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
23
Striatonigral degeneration with motor neuron disease. ( 10896276 )
2000
24
An early description of striatonigral degeneration. ( 10836627 )
2000
25
In vivo magnetic resonance imaging of embryonic neural grafts in a rat model of striatonigral degeneration (multiple system atrophy). ( 10913326 )
2000
26
Failure of neuroprotection by embryonic striatal grafts in a double lesion rat model of striatonigral degeneration (multiple system atrophy). ( 10877927 )
2000
27
Neuropsychological follow up in patients with Parkinson's disease, striatonigral degeneration-type multisystem atrophy, and progressive supranuclear palsy. ( 10945805 )
2000
28
Complex motor disturbances in a sequential double lesion rat model of striatonigral degeneration (multiple system atrophy). ( 10924951 )
2000
29
Loss of dopamine uptake sites and dopamine D2 receptors in striatonigral degeneration. ( 10661519 )
2000
30
An early report of striatonigral degeneration. ( 10634258 )
2000
31
An early description of striatonigral degeneration. ( 10431772 )
1999
32
Differentiation of idiopathic Parkinson's disease from striatonigral degeneration and progressive supranuclear palsy using iodine-123 meta-iodobenzylguanidine myocardial scintigraphy. ( 9562324 )
1998
33
Cortical and brain stem hyperexcitability in striatonigral degeneration. ( 9613764 )
1998
34
Increased basal ganglia iron in striatonigral degeneration: in vivo estimation with magnetic resonance. ( 9532280 )
1998
35
Medial pallidotomy in late-stage Parkinson's disease and striatonigral degeneration. ( 9348415 )
1997
36
Urinary disturbances in striatonigral degeneration and Parkinson's disease: clinical and urodynamic aspects. ( 9251068 )
1997
37
Correlative studies of MR findings with neuropathology in Shy-Drager syndrome and striatonigral degeneration. ( 9594269 )
1997
38
MRI in sporadic olivopontocerebellar atrophy and striatonigral degeneration. ( 9065575 )
1997
39
Subregional loss of putaminal efferents to the basal ganglia output nuclei may cause parkinsonism in striatonigral degeneration. ( 8857740 )
1996
40
[Axial (neck and trunk) rigidity in Parkinson's disease, striatonigral degeneration and progressive supranuclear palsy]. ( 8997136 )
1996
41
Reversal of behavioural abnormalities by fetal allografts in a novel rat model of striatonigral degeneration. ( 8866493 )
1996
42
"Pure" striatonigral degeneration and Parkinson's disease: a comparative clinical study. ( 7651445 )
1995
43
Striatal opioid receptor binding in Parkinson's disease, striatonigral degeneration and Steele-Richardson-Olszewski syndrome, A [11C]diprenorphine PET study. ( 7655890 )
1995
44
Neuropsychological pattern of striatonigral degeneration: comparison with Parkinson's disease and progressive supranuclear palsy. ( 7876847 )
1995
45
Striatonigral degeneration combined with olivopontocerebellar atrophy with subcortical dementia and hallucinatory state. ( 7550604 )
1995
46
Neuroleptic malignant syndrome in striatonigral degeneration. ( 8554665 )
1995
47
Striatonigral degeneration: iron deposition in putamen correlates with the slit-like void signal of magnetic resonance imaging. ( 7533048 )
1994
48
Striatonigral degeneration with neurofibrillary tangles. ( 8256593 )
1993
49
Striatal hypometabolism distinguishes striatonigral degeneration from Parkinson's disease. ( 8498828 )
1993
50
Dopamine D1 receptors in Parkinson's disease and striatonigral degeneration: a positron emission tomography study. ( 8505636 )
1993

Variations for Striatonigral Degeneration

Expression for Striatonigral Degeneration

Search GEO for disease gene expression data for Striatonigral Degeneration.

Pathways for Striatonigral Degeneration

Pathways related to Striatonigral Degeneration according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.88 SLC6A3 SNCA
2 10.13 SLC6A3 SNCA

GO Terms for Striatonigral Degeneration

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

# Name GO ID Score Top Affiliating Genes
1 neuronal cell body GO:0043025 9.54 MAP2 SLC6A3 SNCA
2 axon GO:0030424 9.5 DRD2 SLC6A3 SNCA
3 synaptic vesicle membrane GO:0030672 9.43 DRD2 SNCA
4 GABA-ergic synapse GO:0098982 9.4 ATP2B3 DRD2
5 integral component of presynaptic membrane GO:0099056 9.26 DRD2 SLC6A3
6 axon terminus GO:0043679 9.16 DRD2 SNCA
7 integral component of postsynaptic membrane GO:0099055 8.96 DRD2 SLC6A3
8 dopaminergic synapse GO:0098691 8.62 DRD2 SLC6A3

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 response to drug GO:0042493 9.7 DRD2 SLC6A3 SNCA
2 negative regulation of protein phosphorylation GO:0001933 9.55 DRD2 SNCA
3 excitatory postsynaptic potential GO:0060079 9.54 DRD2 SNCA
4 response to nicotine GO:0035094 9.51 DRD2 SLC6A3
5 positive regulation of multicellular organism growth GO:0040018 9.49 DRD2 SLC6A3
6 regulation of long-term neuronal synaptic plasticity GO:0048169 9.48 DRD2 SNCA
7 behavioral response to cocaine GO:0048148 9.46 DRD2 SNCA
8 response to iron ion GO:0010039 9.43 DRD2 SLC6A3
9 synaptic transmission, dopaminergic GO:0001963 9.4 DRD2 SNCA
10 dopamine metabolic process GO:0042417 9.37 DRD2 SNCA
11 prepulse inhibition GO:0060134 9.32 DRD2 SLC6A3
12 negative regulation of microtubule polymerization GO:0031115 9.26 MAP2 SNCA
13 dopamine biosynthetic process GO:0042416 9.16 SLC6A3 SNCA
14 adenohypophysis development GO:0021984 8.96 DRD2 SLC6A3
15 response to cocaine GO:0042220 8.8 DRD2 SLC6A3 SNCA

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

# Name GO ID Score Top Affiliating Genes
1 drug binding GO:0008144 9.26 DRD2 SLC6A3
2 kinesin binding GO:0019894 9.16 NUP62 SNCA
3 Hsp70 protein binding GO:0030544 8.96 NUP62 SNCA
4 dopamine binding GO:0035240 8.62 DRD2 SLC6A3

Sources for Striatonigral Degeneration

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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