MCID: CRB033
MIFTS: 42

Cerebral Degeneration

Categories: Neuronal diseases

Aliases & Classifications for Cerebral Degeneration

MalaCards integrated aliases for Cerebral Degeneration:

Name: Cerebral Degeneration 12 15
Degenerative Brain Disorder 72
Brain Degeneration 12

Classifications:



External Ids:

Disease Ontology 12 DOID:1443
ICD9CM 35 331.9
SNOMED-CT 68 52522001 73768007
UMLS 72 C0154671

Summaries for Cerebral Degeneration

Disease Ontology : 12 A brain disease that is characterized by loss of structure or function of neurons, including death of neurons and loss of brain tissue.

MalaCards based summary : Cerebral Degeneration, also known as degenerative brain disorder, is related to leukodystrophy and aceruloplasminemia. An important gene associated with Cerebral Degeneration is NOTCH3 (Notch Receptor 3), and among its related pathways/superpathways is Peroxisome. The drugs Galantamine and Neurotransmitter Agents have been mentioned in the context of this disorder. Affiliated tissues include brain, liver and skin, and related phenotypes are no effect and behavior/neurological

Wikipedia : 75 Neurodegeneration is the progressive loss of structure or function of neurons, including death of... more...

Related Diseases for Cerebral Degeneration

Diseases related to Cerebral Degeneration via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 82)
# Related Disease Score Top Affiliating Genes
1 leukodystrophy 30.7 POLR3B POLR3A PLP1 GJC2 GFAP EIF2B5
2 aceruloplasminemia 30.0 POLG PLA2G6 GFAP
3 mitochondrial dna depletion syndrome 4a 11.5
4 norrie disease 11.2
5 niemann-pick disease 11.1
6 brain small vessel disease 1 with or without ocular anomalies 11.1
7 brain small vessel disease 2 11.1
8 hydrocephalus 11.1
9 cerebrotendinous xanthomatosis 10.9
10 canavan disease 10.9
11 lipid storage disease 10.9
12 pelizaeus-merzbacher-like disease 10.5 PLP1 GJC2
13 inflammatory mfh 10.5 SERPINA3 GFAP
14 adrenomyeloneuropathy 10.5 SLC25A17 ABCD1
15 leukodystrophy, hypomyelinating, 4 10.5 PLP1 GJC2
16 binswanger's disease 10.5 PLP1 NOTCH3 GFAP
17 spastic paraplegia 2, x-linked 10.5 PLP1 GJC2
18 spinocerebellar degeneration 10.4 POLG ABCD1
19 demyelinating disease 10.4 PLP1 ABCD2 ABCD1
20 adrenoleukodystrophy 10.3 ABCD3 ABCD2 ABCD1
21 cortical blindness 10.3 POLG ABCD1
22 hereditary spastic paraplegia 10.3 PLP1 PLA2G6 L1CAM GJC2
23 hypomyelinating leukoencephalopathy 10.2 POLR3B POLR3A PLP1 GJC2
24 hypomyelinating leukodystrophy 10.2 POLR3B POLR3A PLP1 GJC2
25 granular cell tumor 10.1 SERPINA3 GFAP
26 x-linked recessive disease 10.0 SLC25A17 PLP1 ABCD3 ABCD2 ABCD1
27 amyotrophic lateral sclerosis 1 10.0
28 lateral sclerosis 10.0
29 encephalopathy 9.9
30 apraxia 9.8
31 polg-related disorders 9.8
32 cerebral atrophy 9.8
33 ataxia and polyneuropathy, adult-onset 9.8
34 microhydranencephaly 9.8
35 dementia 9.8
36 autosomal dominant cerebellar ataxia 9.8
37 fetal brain disruption sequence 9.8
38 alzheimer disease 9.7
39 mitochondrial complex iv deficiency 9.7
40 aging 9.7
41 yemenite deaf-blind hypopigmentation syndrome 9.7
42 aphasia 9.7
43 split hand-foot malformation 9.7
44 hemiplegia 9.7
45 bronchopneumonia 9.7
46 leukemia 9.7
47 hepatic coma 9.7
48 acute leukemia 9.7
49 hepatic encephalopathy 9.7
50 kluver-bucy syndrome 9.7

Comorbidity relations with Cerebral Degeneration via Phenotypic Disease Network (PDN): (show all 24)


Acute Cystitis Alzheimer Disease
Bronchitis Cerebral Atherosclerosis
Cerebrovascular Disease Decubitus Ulcer
Deficiency Anemia Encephalopathy
Epilepsy, Focal, with Speech Disorder and with or Without Mental Retardation Familial Atrial Fibrillation
Generalized Atherosclerosis Heart Disease
Hypertension, Essential Hypothyroidism
Iron Deficiency Anemia Major Depressive Disorder
Obstructive Hydrocephalus Osteoporosis
Parkinson Disease, Late-Onset Protein-Energy Malnutrition
Schizophreniform Disorder Spinocerebellar Degeneration
Swallowing Disorders Transient Cerebral Ischemia

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



Diseases related to Cerebral Degeneration

Symptoms & Phenotypes for Cerebral Degeneration

GenomeRNAi Phenotypes related to Cerebral Degeneration according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 no effect GR00402-S-1 9.92 ABCD1 ABCD2 ABCD3 ASPA DCAF8 EIF2B2

MGI Mouse Phenotypes related to Cerebral Degeneration:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.06 ABCD1 ABCD2 ASPA EIF2B5 GFAP GJC2
2 hematopoietic system MP:0005397 10 ABCD1 ABCD2 ASPA EIF2B2 EIF2B5 GJC2
3 immune system MP:0005387 9.85 ABCD1 ABCD2 EIF2B5 GFAP GJC2 NOTCH3
4 nervous system MP:0003631 9.77 ABCD1 ABCD2 ASPA EIF2B5 GFAP GJC2
5 vision/eye MP:0005391 9.32 ABCD1 ASPA EIF2B5 GFAP GJC2 L1CAM

Drugs & Therapeutics for Cerebral Degeneration

Drugs for Cerebral Degeneration (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 20)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Galantamine Approved Phase 3 357-70-0 9651
2 Neurotransmitter Agents Phase 3
3 Cholinergic Agents Phase 3
4 Peripheral Nervous System Agents Phase 3
5 Cholinesterase Inhibitors Phase 3
6 Nootropic Agents Phase 3
7 Autonomic Agents Phase 3
8
Montelukast Approved Phase 2 158966-92-8 5281040
9 Respiratory System Agents Phase 2
10 Cytochrome P-450 Enzyme Inducers Phase 2
11 Hormones Phase 2
12 Hormone Antagonists Phase 2
13 Anti-Asthmatic Agents Phase 2
14 Leukotriene Antagonists Phase 2
15 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
16
Triamcinolone Approved, Vet_approved 124-94-7 31307
17 triamcinolone acetonide
18 Triamcinolone diacetate
19 Triamcinolone hexacetonide
20 Fluorodeoxyglucose F18

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A European Multi-Center Study to Determine the Safety and Efficacy of Galanthamine Hydrobromide 40mg/Day (32 mg /Day GAL Base, Tid Dose Regimen) in Patients Diagnosed With Alzheimer-Type Dementia Completed NCT00338117 Phase 3 Galantamine hydrobromide
2 Effects of Montelukast Therapy on Alzheimer's Disease (EMERALD) Not yet recruiting NCT03991988 Phase 2 Montelukast;Placebo oral tablet
3 Magnetic Resonance Imaging Biomarkers in Amyotrophic Lateral Sclerosis Completed NCT02405182
4 The Motor Network in Essential Tremor: Mechanisms of Therapy Recruiting NCT02712515
5 Closing the Loop on Tremor: A Responsive Deep Brain Stimulator for the Treatment of Essential Tremor Recruiting NCT02649166
6 Application of Transcranial Alternating Current Stimulation for Modulation of Sleep and Cognitive Performance Recruiting NCT03112902
7 Novel MRI Biomarkers for Monitoring Disease Progression in ALS Recruiting NCT03362658
8 Coaching for Cognition in Alzheimer's (COCOA) Recruiting NCT03424200
9 Benefit of MRI and 18F-FDG PET Imaging in the Early Diagnosis of Alzheimer-like Dementia Terminated NCT01815112
10 Treating Sleep Wake Cycle Disturbances in Basal Ganglia Neurodegenerative Disorder Subjects With Ramelteon- A Double Blind, Placebo Controlled Trial Withdrawn NCT00907595 Ramelteon;Placebo

Search NIH Clinical Center for Cerebral Degeneration

Genetic Tests for Cerebral Degeneration

Anatomical Context for Cerebral Degeneration

MalaCards organs/tissues related to Cerebral Degeneration:

41
Brain, Liver, Skin, Testes, Cortex, Amygdala, Breast

Publications for Cerebral Degeneration

Articles related to Cerebral Degeneration:

(show top 50) (show all 177)
# Title Authors PMID Year
1
Quantifying changes on susceptibility weighted images in amyotrophic lateral sclerosis using MRI texture analysis. 38
31025885 2019
2
Safety, Tolerability, Pharmacokinetics, and Pharmacodynamic Effects of PF-06751979, a Potent and Selective Oral BACE1 Inhibitor: Results from Phase I Studies in Healthy Adults and Healthy Older Subjects. 38
31424395 2019
3
Reliability of 3D texture analysis: A multicenter MRI study of the brain. 38
31423714 2019
4
Management of concussion in soccer. 38
30687899 2019
5
Alabama Brief Cognitive Screener: Utility of a New Cognitive Screening Instrument in a Memory Disorders Clinic. 38
30896091 2019
6
A study of the effects of 8-week acupuncture treatment on patients with Parkinson's disease. 38
30557997 2018
7
Evaluating the cerebral correlates of survival in amyotrophic lateral sclerosis. 38
30480029 2018
8
Texture Analysis to Detect Cerebral Degeneration in Amyotrophic Lateral Sclerosis. 38
30234470 2018
9
The role of Alu-derived RNAs in Alzheimer's and other neurodegenerative conditions. 38
29685192 2018
10
Interaction between the AAA ATPase p97/VCP and a concealed UBX domain in the copper transporter ATP7A is associated with motor neuron degeneration. 38
29599289 2018
11
A strategy to find novel candidate anti-Alzheimer's disease drugs by constructing interaction networks between drug targets and natural compounds in medical plants. 38
29770277 2018
12
Type-I interferons in Parkinson's disease: innate inflammatory response drives fate of neurons in model of degenerative brain disorder: An editorial comment on 'Type-I interferons mediate the neuroinflammatory response and neurotoxicity induced by rotenone'. 38
28332229 2017
13
Widespread temporo-occipital lobe dysfunction in amyotrophic lateral sclerosis. 38
28067298 2017
14
Abnormal Brain Connectivity Spectrum Disorders Following Thimerosal Administration: A Prospective Longitudinal Case-Control Assessment of Medical Records in the Vaccine Safety Datalink. 38
28539852 2017
15
Clinicopathological characteristics of patients with amyotrophic lateral sclerosis resulting in a totally locked-in state (communication Stage V). 38
27716404 2016
16
Dispersion entropy for the analysis of resting-state MEG regularity in Alzheimer's disease. 38
28269715 2016
17
Crosstalk between 6-OHDA-induced autophagy and apoptosis in PC12 cells is mediated by phosphorylation of Raf-1/ERK1/2. 38
29442034 2016
18
Split Hand / Foot Malformation Syndrome with Cerebral Degeneration. 38
27730782 2016
19
High Content, Multi-Parameter Analyses in Buccal Cells to Identify Alzheimer's Disease. 38
26975368 2016
20
Cerebral Degeneration in Amyotrophic Lateral Sclerosis Revealed by 3-Dimensional Texture Analysis. 38
27064416 2016
21
Thimerosal exposure and increased risk for diagnosed tic disorder in the United States: a case-control study. 38
27486363 2015
22
Early detection of widespread progressive brain injury after cardiac arrest: a single case DTI and post-mortem histology study. 38
24633135 2014
23
[Genetic aspects of Alzheimer's disease (Review)]. 38
24035416 2013
24
Improved prediction of Alzheimer's disease with longitudinal white matter/gray matter contrast changes. 38
22674625 2013
25
Specific needs of dopamine neurons for stimulation in order to survive: implication for Parkinson disease. 38
23699175 2013
26
Mentalizing in preclinical Huntington's disease: an fMRI study using cartoon picture stories. 38
23179063 2013
27
Mitochondrial aldehyde dehydrogenase-2 activation prevents β-amyloid-induced endothelial cell dysfunction and restores angiogenesis. 38
23447675 2013
28
Phenotypic spectrum of COL4A1 mutations: porencephaly to schizencephaly. 38
23225343 2013
29
The response of cerebral cortex to haemorrhagic damage: experimental evidence from a penetrating injury model. 38
23555765 2013
30
Disease-specific expression of the serotonin-receptor 5-HT(2C) in natural killer cells in Alzheimer's dementia. 38
22766135 2012
31
Acupuncture stimulation on GB34 activates neural responses associated with Parkinson's disease. 38
22943145 2012
32
Fractional anisotropy of water diffusion in cerebral white matter across the lifespan. 38
20122755 2012
33
De novo and inherited mutations in COL4A2, encoding the type IV collagen α2 chain cause porencephaly. 38
22209246 2012
34
Intranasal treatment of neurodegenerative diseases and stroke. 38
22202044 2012
35
Transient improvement of acquired hepatocerebral degeneration with parkinsonian symptoms after failed liver transplant: case report and literature review. 38
22142042 2011
36
The multiple phenotypes of corticobasal syndrome and corticobasal degeneration: implications for further study. 38
21853287 2011
37
Association between environmental exposure to pesticides and neurodegenerative diseases. 38
21601587 2011
38
The extrinsic and intrinsic apoptotic pathways are involved in manganese toxicity in rat astrocytoma C6 cells. 38
21704098 2011
39
Punica granatum protects against oxidative stress in PC12 cells and oxidative stress-induced Alzheimer's symptoms in mice. 38
21631359 2011
40
"Theory of mind" is impaired in Huntington's disease. 38
21384426 2011
41
Visual signs and symptoms of progressive supranuclear palsy. 38
20629667 2011
42
Xanthene food dye, as a modulator of Alzheimer's disease amyloid-beta peptide aggregation and the associated impaired neuronal cell function. 38
21998691 2011
43
Impulsive behavior and associated clinical variables in Parkinson's disease. 38
21300194 2011
44
Progression of brain atrophy and cognitive decline in diabetes mellitus: a 3-year follow-up. 38
20837967 2010
45
Proteomic analysis of aging brain in SAMP10 mouse: a model of age-related cerebral degeneration. 38
20471994 2010
46
Amygdala size reduction is associated with memory deficits in complicated hereditary spastic paraparesis: an MRI study. 38
20664204 2010
47
Are astrocytes the missing link between lack of brain aspartoacylase activity and the spongiform leukodystrophy in Canavan disease? 38
19319678 2009
48
The role of stress and anxiety in the onset of familial Creutzfeldt-Jakob Disease (CJD): review. 38
19051123 2009
49
Methodology for discovery of Alzheimer's disease blood-based biomarkers. 38
19366883 2009
50
Novel anti-Alzheimer's dimer Bis(7)-cognitin: cellular and molecular mechanisms of neuroprotection through multiple targets. 38
19110209 2009

Variations for Cerebral Degeneration

Expression for Cerebral Degeneration

Search GEO for disease gene expression data for Cerebral Degeneration.

Pathways for Cerebral Degeneration

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

# Super pathways Score Top Affiliating Genes
1 10.84 SLC25A17 ABCD3 ABCD2 ABCD1

GO Terms for Cerebral Degeneration

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

# Name GO ID Score Top Affiliating Genes
1 myelin sheath GO:0043209 9.4 PLP1 GJC2
2 RNA polymerase III complex GO:0005666 9.37 POLR3B POLR3A
3 integral component of peroxisomal membrane GO:0005779 9.26 SLC25A17 ABCD1
4 peroxisome GO:0005777 9.26 SLC25A17 ABCD3 ABCD2 ABCD1
5 eukaryotic translation initiation factor 2B complex GO:0005851 9.16 EIF2B5 EIF2B2
6 peroxisomal membrane GO:0005778 8.92 SLC25A17 ABCD3 ABCD2 ABCD1
7 cytosol GO:0005829 10.1 POLR3B POLR3A PLA2G6 NOTCH3 GFAP EIF2B5

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

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 transmembrane transport GO:0055085 9.92 SLC25A17 GJC2 ABCD3 ABCD2 ABCD1
2 myelination GO:0042552 9.61 PLP1 EIF2B5 EIF2B2
3 positive regulation of interferon-beta production GO:0032728 9.57 POLR3B POLR3A
4 oligodendrocyte development GO:0014003 9.56 EIF2B5 EIF2B2
5 peroxisome organization GO:0007031 9.55 ABCD3 ABCD1
6 negative regulation of cytokine production involved in inflammatory response GO:1900016 9.54 ABCD1 ABCD2
7 negative regulation of reactive oxygen species biosynthetic process GO:1903427 9.51 ABCD2 ABCD1
8 central nervous system myelination GO:0022010 9.49 PLP1 ASPA
9 positive regulation of fatty acid beta-oxidation GO:0032000 9.48 ABCD2 ABCD1
10 neuron projection maintenance GO:1990535 9.46 ABCD2 ABCD1
11 astrocyte development GO:0014002 9.43 PLP1 GFAP EIF2B5
12 myelin maintenance GO:0043217 9.4 ABCD2 ABCD1
13 positive regulation of unsaturated fatty acid biosynthetic process GO:2001280 9.37 ABCD2 ABCD1
14 peroxisomal long-chain fatty acid import GO:0015910 9.32 ABCD3 ABCD1
15 fatty acid beta-oxidation GO:0006635 9.26 SLC25A17 ABCD3 ABCD2 ABCD1
16 axon development GO:0061564 9.16 PLP1 L1CAM
17 very long-chain fatty acid catabolic process GO:0042760 8.8 ABCD3 ABCD2 ABCD1

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

# Name GO ID Score Top Affiliating Genes
1 nucleotidyltransferase activity GO:0016779 9.33 POLR3B POLR3A POLG
2 RNA polymerase III activity GO:0001056 9.26 POLR3B POLR3A
3 ATPase activity, coupled to transmembrane movement of substances GO:0042626 9.13 ABCD3 ABCD2 ABCD1
4 long-chain fatty acid transporter activity GO:0005324 8.62 ABCD3 ABCD1

Sources for Cerebral Degeneration

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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