MCID: STR001
MIFTS: 38

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 55 56 45 15 74

Classifications:



External Ids:

Disease Ontology 12 DOID:4751
MeSH 45 D020955
NCIt 51 C125695
SNOMED-CT 69 29618004
ICD10 34 G23.2
UMLS 74 C0270733

Summaries for Striatonigral Degeneration

NINDS : 55 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 supranuclear palsy, progressive, 1 and multiple system atrophy 1. An important gene associated with Striatonigral Degeneration is VAC14 (VAC14 Component Of PIKFYVE Complex), and among its related pathways/superpathways are cAMP signaling pathway and Microglia Activation During Neuroinflammation: Steady-State Microglia. Affiliated tissues include brain, cerebellum and medulla oblongata, and related phenotypes are behavior/neurological and mortality/aging

Wikipedia : 77 Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by autonomic... 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 47)
# Related Disease Score Top Affiliating Genes
1 supranuclear palsy, progressive, 1 30.9 SLC6A3 SNCA
2 multiple system atrophy 1 30.8 DRD2 SLC6A3 SNCA
3 olivopontocerebellar atrophy 30.0 ATP2B3 MAP2 SNCA
4 dystonia 29.9 DRD2 SLC6A3 VAC14
5 parkinson disease, late-onset 29.5 DRD1 DRD2 SLC6A3 SNCA
6 striatonigral degeneration, infantile 12.7
7 striatonigral degeneration, childhood-onset 12.6
8 striatonigral degeneration, infantile, mitochondrial 12.4
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 rem sleep behavior disorder 10.1 SLC6A3 SNCA
16 tic disorder 10.1 DRD2 SLC6A3
17 early-onset schizophrenia 10.1 DRD1 DRD2
18 alcohol-induced mental disorder 10.1 DRD2 SLC6A3
19 motor neuron disease 10.1
20 corticobasal degeneration 10.1
21 dementia - subcortical 10.1
22 alcoholic psychosis 10.1 DRD2 SLC6A3
23 oppositional defiant disorder 10.1 DRD2 SLC6A3
24 antisocial personality disorder 10.1 DRD2 SLC6A3
25 perry syndrome 10.1 SLC6A3 SNCA
26 personality disorder 10.1 DRD2 SLC6A3
27 stuttering 10.1 DRD2 SLC6A3
28 restless legs syndrome 10.0 DRD2 SLC6A3
29 essential tremor 10.0 SLC6A3 SNCA
30 cocaine dependence 10.0 DRD2 SLC6A3
31 conduct disorder 10.0 DRD2 SLC6A3
32 machado-joseph disease 10.0 SLC6A3 SNCA
33 substance abuse 9.9 DRD2 SLC6A3
34 eating disorder 9.9 DRD2 SLC6A3
35 cocaine abuse 9.9 DRD2 SLC6A3 SNCA
36 movement disease 9.9 DRD2 SLC6A3 SNCA
37 disease of mental health 9.9 DRD2 SLC6A3 SNCA
38 alcohol abuse 9.8 DRD1 DRD2
39 pathological gambling 9.8 DRD1 DRD2 SLC6A3
40 psychotic disorder 9.8 DRD1 DRD2 SLC6A3
41 obsessive-compulsive disorder 9.8 DRD1 DRD2 SLC6A3
42 alcohol dependence 9.8 DRD1 DRD2 SLC6A3
43 attention deficit-hyperactivity disorder 9.8 DRD1 DRD2 SLC6A3
44 transient cerebral ischemia 9.7 DRD1 MAP2
45 substance dependence 9.7 DRD2 SLC6A3
46 toxic encephalopathy 9.7 MAP2 SLC6A3 SNCA
47 schizophrenia 9.3 DRD1 DRD2 MAP2 SLC6A3

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:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.8 DRD1 DRD2 MAP2 SLC6A3 SNCA VAC14
2 mortality/aging MP:0010768 9.7 DRD1 DRD2 MAP2 NUP62 SLC6A3 SNCA
3 integument MP:0010771 9.65 DRD1 DRD2 SLC6A3 SNCA VAC14
4 nervous system MP:0003631 9.43 DRD1 DRD2 MAP2 SLC6A3 SNCA VAC14
5 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:

42
Brain, Cerebellum, Medulla Oblongata, Eye

Publications for Striatonigral Degeneration

Articles related to Striatonigral Degeneration:

(show top 50) (show all 83)
# Title Authors Year
1
Progressive striatonigral degeneration in a transgenic mouse model of multiple system atrophy: translational implications for interventional therapies. ( 29298733 )
2018
2
Early Motor Fluctuations in a Patient with Striatonigral Degeneration. ( 28101035 )
2016
3
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
4
Intrastriatal injection of colchicine induces striatonigral degeneration in mice. ( 18564367 )
2008
5
Effects of riluzole on combined MPTP + 3-nitropropionic acid-induced mild to moderate striatonigral degeneration in mice. ( 15503195 )
2005
6
Failure of caspase inhibition in the double-lesion rat model of striatonigral degeneration (multiple system atrophy). ( 15549330 )
2005
7
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
8
MPTP potentiates 3-nitropropionic acid-induced striatal damage in mice: reference to striatonigral degeneration. ( 14697318 )
2004
9
Effects of pulsatile L-DOPA treatment in the double lesion rat model of striatonigral degeneration (multiple system atrophy). ( 15056471 )
2004
10
Failure of neuronal protection by inhibition of glial activation in a rat model of striatonigral degeneration. ( 15372496 )
2004
11
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
12
Thin section MR study of the basal ganglia in the differential diagnosis between striatonigral degeneration and Parkinson disease. ( 11884785 )
2002
13
Patterns of protein nitration in dementia with Lewy bodies and striatonigral degeneration. ( 11935266 )
2002
14
A novel grading scale for striatonigral degeneration (multiple system atrophy). ( 11956953 )
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 'single toxin-double lesion' rat model of striatonigral degeneration by intrastriatal 1-methyl-4-phenylpyridinium ion injection: a motor behavioural analysis. ( 12421620 )
2002
17
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
18
Simultaneous intrastriatal 6-hydroxydopamine and quinolinic acid injection: a model of early-stage striatonigral degeneration. ( 11161601 )
2001
19
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
20
FLAIR MRI of striatonigral degeneration. ( 11342686 )
2001
21
Visual event-related potentials in progressive supranuclear palsy, corticobasal degeneration, striatonigral degeneration, and Parkinson's disease. ( 10896267 )
2000
22
Striatonigral degeneration with motor neuron disease. ( 10896276 )
2000
23
An early report of striatonigral degeneration. ( 10634258 )
2000
24
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
25
Loss of dopamine uptake sites and dopamine D2 receptors in striatonigral degeneration. ( 10661519 )
2000
26
Toward a primate model of L-dopa-unresponsive parkinsonism mimicking striatonigral degeneration. ( 10830420 )
2000
27
An early description of striatonigral degeneration. ( 10836627 )
2000
28
Failure of neuroprotection by embryonic striatal grafts in a double lesion rat model of striatonigral degeneration (multiple system atrophy). ( 10877927 )
2000
29
In vivo magnetic resonance imaging of embryonic neural grafts in a rat model of striatonigral degeneration (multiple system atrophy). ( 10913326 )
2000
30
Complex motor disturbances in a sequential double lesion rat model of striatonigral degeneration (multiple system atrophy). ( 10924951 )
2000
31
Neuropsychological follow up in patients with Parkinson's disease, striatonigral degeneration-type multisystem atrophy, and progressive supranuclear palsy. ( 10945805 )
2000
32
An early description of striatonigral degeneration. ( 10431772 )
1999
33
Increased basal ganglia iron in striatonigral degeneration: in vivo estimation with magnetic resonance. ( 9532280 )
1998
34
Differentiation of idiopathic Parkinson's disease from striatonigral degeneration and progressive supranuclear palsy using iodine-123 meta-iodobenzylguanidine myocardial scintigraphy. ( 9562324 )
1998
35
Cortical and brain stem hyperexcitability in striatonigral degeneration. ( 9613764 )
1998
36
MRI in sporadic olivopontocerebellar atrophy and striatonigral degeneration. ( 9065575 )
1997
37
Urinary disturbances in striatonigral degeneration and Parkinson's disease: clinical and urodynamic aspects. ( 9251068 )
1997
38
Medial pallidotomy in late-stage Parkinson's disease and striatonigral degeneration. ( 9348415 )
1997
39
Correlative studies of MR findings with neuropathology in Shy-Drager syndrome and striatonigral degeneration. ( 9594269 )
1997
40
Subregional loss of putaminal efferents to the basal ganglia output nuclei may cause parkinsonism in striatonigral degeneration. ( 8857740 )
1996
41
Reversal of behavioural abnormalities by fetal allografts in a novel rat model of striatonigral degeneration. ( 8866493 )
1996
42
[Axial (neck and trunk) rigidity in Parkinson's disease, striatonigral degeneration and progressive supranuclear palsy]. ( 8997136 )
1996
43
Neuroleptic malignant syndrome in striatonigral degeneration. ( 8554665 )
1995
44
Striatonigral degeneration combined with olivopontocerebellar atrophy with subcortical dementia and hallucinatory state. ( 7550604 )
1995
45
"Pure" striatonigral degeneration and Parkinson's disease: a comparative clinical study. ( 7651445 )
1995
46
Striatal opioid receptor binding in Parkinson's disease, striatonigral degeneration and Steele-Richardson-Olszewski syndrome, A [11C]diprenorphine PET study. ( 7655890 )
1995
47
Neuropsychological pattern of striatonigral degeneration: comparison with Parkinson's disease and progressive supranuclear palsy. ( 7876847 )
1995
48
Striatonigral degeneration: iron deposition in putamen correlates with the slit-like void signal of magnetic resonance imaging. ( 7533048 )
1994
49
Striatonigral degeneration with neurofibrillary tangles. ( 8256593 )
1993
50
Dopamine D1 and D2 receptors in Parkinson's disease and striatonigral degeneration determined by PET. ( 8420176 )
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 11.66 ATP2B3 DRD1 DRD2
2 10.97 DRD1 DRD2
3
Show member pathways
10.87 DRD1 DRD2 SLC6A3
4 10.76 SLC6A3 SNCA
5 10.3 DRD1 DRD2

GO Terms for Striatonigral Degeneration

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 neuronal cell body GO:0043025 9.65 MAP2 SLC6A3 SNCA
2 axon GO:0030424 9.63 DRD2 SLC6A3 SNCA
3 glutamatergic synapse GO:0098978 9.58 ATP2B3 DRD1 DRD2
4 synaptic vesicle membrane GO:0030672 9.49 DRD2 SNCA
5 axon terminus GO:0043679 9.43 DRD2 SNCA
6 ciliary membrane GO:0060170 9.37 DRD1 DRD2
7 GABA-ergic synapse GO:0098982 9.33 ATP2B3 DRD1 DRD2
8 non-motile cilium GO:0097730 9.32 DRD1 DRD2
9 dopaminergic synapse GO:0098691 9.26 DRD2 SLC6A3
10 integral component of presynaptic membrane GO:0099056 9.13 DRD1 DRD2 SLC6A3
11 integral component of postsynaptic membrane GO:0099055 8.8 DRD1 DRD2 SLC6A3

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

(show all 36)
# Name GO ID Score Top Affiliating Genes
1 response to drug GO:0042493 9.76 DRD1 DRD2 SLC6A3 SNCA
2 negative regulation of protein phosphorylation GO:0001933 9.67 DRD2 SNCA
3 excitatory postsynaptic potential GO:0060079 9.67 DRD2 SNCA
4 locomotory behavior GO:0007626 9.67 DRD1 DRD2 SLC6A3
5 protein import into nucleus GO:0006606 9.66 DRD1 NUP62
6 synapse assembly GO:0007416 9.66 DRD1 DRD2
7 regulation of synaptic vesicle exocytosis GO:2000300 9.65 DRD1 DRD2
8 visual learning GO:0008542 9.65 DRD1 DRD2
9 long-term synaptic potentiation GO:0060291 9.65 DRD1 SNCA
10 response to nicotine GO:0035094 9.64 DRD2 SLC6A3
11 positive regulation of multicellular organism growth GO:0040018 9.64 DRD2 SLC6A3
12 response to amphetamine GO:0001975 9.63 DRD1 DRD2
13 feeding behavior GO:0007631 9.63 DRD1 DRD2
14 adult walking behavior GO:0007628 9.62 DRD1 DRD2
15 associative learning GO:0008306 9.62 DRD1 DRD2
16 positive regulation of release of sequestered calcium ion into cytosol GO:0051281 9.61 DRD1 SNCA
17 regulation of long-term neuronal synaptic plasticity GO:0048169 9.61 DRD2 SNCA
18 temperature homeostasis GO:0001659 9.6 DRD1 DRD2
19 dopamine receptor signaling pathway GO:0007212 9.59 DRD1 DRD2
20 response to iron ion GO:0010039 9.58 DRD2 SLC6A3
21 striatum development GO:0021756 9.58 DRD1 DRD2
22 grooming behavior GO:0007625 9.57 DRD1 DRD2
23 negative regulation of microtubule polymerization GO:0031115 9.56 MAP2 SNCA
24 dopamine biosynthetic process GO:0042416 9.55 SLC6A3 SNCA
25 peristalsis GO:0030432 9.54 DRD1 DRD2
26 regulation of dopamine metabolic process GO:0042053 9.52 DRD1 SLC6A3
27 adenohypophysis development GO:0021984 9.51 DRD2 SLC6A3
28 behavioral response to cocaine GO:0048148 9.5 DRD1 DRD2 SNCA
29 phospholipase C-activating dopamine receptor signaling pathway GO:0060158 9.48 DRD1 DRD2
30 cerebral cortex GABAergic interneuron migration GO:0021853 9.43 DRD1 DRD2
31 synaptic transmission, dopaminergic GO:0001963 9.43 DRD1 DRD2 SNCA
32 dopamine transport GO:0015872 9.4 DRD1 SLC6A3
33 regulation of dopamine uptake involved in synaptic transmission GO:0051584 9.37 DRD1 DRD2
34 dopamine metabolic process GO:0042417 9.33 DRD1 DRD2 SNCA
35 prepulse inhibition GO:0060134 9.13 DRD1 DRD2 SLC6A3
36 response to cocaine GO:0042220 8.92 DRD1 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 9.32 DRD2 SLC6A3
2 kinesin binding GO:0019894 9.26 NUP62 SNCA
3 Hsp70 protein binding GO:0030544 9.16 NUP62 SNCA
4 dopamine neurotransmitter receptor activity GO:0004952 8.96 DRD1 DRD2
5 dopamine binding GO:0035240 8.8 DRD1 DRD2 SLC6A3

Sources for Striatonigral Degeneration

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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