MCID: CRB033
MIFTS: 42

Cerebral Degeneration

Categories: Neuronal diseases

Aliases & Classifications for Cerebral Degeneration

MalaCards integrated aliases for Cerebral Degeneration:

Name: Cerebral Degeneration 12 29 15
Degenerative Brain Disorder 73
Brain Degeneration 12

Classifications:



Summaries for Cerebral Degeneration

MalaCards based summary : Cerebral Degeneration, also known as degenerative brain disorder, is related to hydrocephalus and leukodystrophy. An important gene associated with Cerebral Degeneration is NOTCH3 (Notch 3), and among its related pathways/superpathways are Neuroscience and Thyroid hormone signaling pathway. The drugs Triamcinolone and Gastrointestinal Agents have been mentioned in the context of this disorder. Affiliated tissues include brain, liver and cortex, and related phenotypes are Decreased viability and Decreased viability

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 52)
# Related Disease Score Top Affiliating Genes
1 hydrocephalus 31.7 GFAP L1CAM PIK3R2 ZIC3
2 leukodystrophy 30.5 ASPA EIF2B2 EIF2B5 FA2H GJC2 PLP1
3 mitochondrial dna depletion syndrome 4a 11.4
4 norrie disease 10.9
5 pseudobulbar palsy 10.8 NOTCH3 NOTCH4
6 niemann-pick disease 10.8
7 porencephaly 2 10.8
8 adrenomyeloneuropathy 10.7 ABCD1 SLC25A17
9 binswanger's disease 10.6 GFAP NOTCH3 PLP1
10 canavan disease 10.6
11 lipid storage disease 10.6
12 pelizaeus-merzbacher-like disease 10.5 GJC2 PLP1
13 megalencephaly 10.5 EIF2B5 MLC1 PIK3R2
14 pelizaeus-merzbacher disease 10.5 GJC2 MLC1 PLP1
15 leukodystrophy, hypomyelinating, 4 10.5 GJC2 PLP1
16 leukodystrophy, hypomyelinating, 2 10.4 FA2H GJC2 PLP1
17 hypomyelinating leukoencephalopathy 10.4 GJC2 PLP1
18 leukoencephalopathy with vanishing white matter 10.4 EIF2B2 EIF2B5 MLC1
19 demyelinating disease 10.4 ABCD1 ABCD2 PLP1
20 cerebritis 10.3
21 spastic paraplegia 2, x-linked 10.3 GJC2 PLP1
22 atypical choroid plexus papilloma 10.2 NOTCH3 NOTCH4
23 adrenoleukodystrophy 10.2 ABCD1 ABCD2 ABCD3 PEX5
24 hereditary spastic paraplegia 10.1 FA2H GJC2 L1CAM PLP1
25 aging 9.9
26 amyotrophic lateral sclerosis 1 9.8
27 lateral sclerosis 9.8
28 neuronitis 9.8
29 trehalase deficiency 9.7 EIF2B5 FA2H GJC2 PIK3R2 PLP1
30 hepatitis 9.7
31 fetal brain disruption sequence 9.7
32 alzheimer disease 9.5
33 acute leukemia 9.5
34 leukemia 9.5
35 liver disease 9.5
36 apraxia 9.5
37 split hand-foot malformation 9.5
38 hemiplegia 9.5
39 bronchopneumonia 9.5
40 dementia 9.5
41 disease of mental health 9.5
42 epilepsy 9.5
43 dysgraphia 9.5
44 herpes simplex 9.5
45 encephalitis 9.5
46 neonatal herpes 9.5
47 encephalopathy 9.5
48 machado-joseph disease 9.5
49 spinocerebellar ataxia 2 9.5
50 wilson disease 9.5

Comorbidity relations with Cerebral Degeneration via Phenotypic Disease Network (PDN): (show all 26)


Acute Cystitis Alzheimer Disease
Bronchitis Cerebral Atherosclerosis
Cerebrovascular Disease Chronic Myocardial Ischemia
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
Ischemic Heart Disease 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 Decreased viability GR00173-A 10.13 PIK3R2
2 Decreased viability GR00221-A-1 10.13 PIK3R2
3 Decreased viability GR00221-A-2 10.13 PIK3R2
4 Decreased viability GR00221-A-4 10.13 PIK3R2
5 Decreased viability GR00240-S-1 10.13 ASAH1
6 Decreased viability GR00301-A 10.13 PIK3R2
7 Decreased viability GR00381-A-1 10.13 EIF2B5 FA2H GFAP ASAH1
8 Decreased viability GR00402-S-2 10.13 FA2H GFAP GJC2 L1CAM MLC1 NOTCH3
9 no effect GR00402-S-1 9.62 ABCD2 ABCD3 ASAH1 ASPA EIF2B2 EIF2B5

MGI Mouse Phenotypes related to Cerebral Degeneration:

46 (show all 12)
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.36 ZIC3 ABCD1 ABCD2 ASAH1 ASPA EIF2B5
2 homeostasis/metabolism MP:0005376 10.28 ABCD1 ABCD2 ABCD3 ASAH1 ASPA EIF2B5
3 growth/size/body region MP:0005378 10.27 ASAH1 ASPA EIF2B5 GFAP L1CAM MLC1
4 hematopoietic system MP:0005397 10.21 ABCD1 ASAH1 ASPA EIF2B2 EIF2B5 GJC2
5 cellular MP:0005384 10.19 ABCD3 ASAH1 GFAP GJC2 L1CAM PLP1
6 nervous system MP:0003631 10.16 ABCD2 ASAH1 ASPA EIF2B5 FA2H GFAP
7 immune system MP:0005387 10.11 NOTCH3 PEX5 PLP1 POLG ZIC3 ABCD1
8 endocrine/exocrine gland MP:0005379 10.08 ABCD1 ABCD2 ASAH1 FA2H MLC1 NOTCH3
9 muscle MP:0005369 9.87 ASPA GFAP NOTCH3 PEX5 PIK3R2 PLP1
10 reproductive system MP:0005389 9.81 ABCD1 ASAH1 L1CAM MLC1 NOTCH3 PEX5
11 skeleton MP:0005390 9.56 ASAH1 ASPA L1CAM NOTCH3 PEX5 PLP1
12 vision/eye MP:0005391 9.32 ABCD1 ASPA FA2H GFAP GJC2 L1CAM

Drugs & Therapeutics for Cerebral Degeneration

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

(show all 12)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Triamcinolone Approved, Vet_approved Not Applicable 124-94-7 31307
2 Gastrointestinal Agents Not Applicable
3 glucagon Not Applicable
4 Glucagon-Like Peptide 1 Not Applicable
5 Hormone Antagonists Not Applicable
6 Hormones Not Applicable
7 Hormones, Hormone Substitutes, and Hormone Antagonists Not Applicable
8 Incretins Not Applicable
9 triamcinolone acetonide Not Applicable
10 Triamcinolone diacetate Not Applicable
11 Triamcinolone hexacetonide Not Applicable
12 Fluorodeoxyglucose F18 Not Applicable

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 MRI Biomarkers in ALS Recruiting NCT02405182
2 To Determine the Effect of GLP1 on Cognitive Function, Brain Glucose Metabolism and Neuroplasticity. Recruiting NCT03414333 Not Applicable GLP-1
3 Application of Transcranial Alternating Current Stimulation for Modulation of Sleep and Cognitive Performance Recruiting NCT03112902 Not Applicable
4 Responsive Deep Brain Stimulator for Essential Tremor Recruiting NCT02649166 Not Applicable
5 Coaching for Cognition in Alzheimer's (COCOA) Recruiting NCT03424200 Not Applicable
6 The Motor Network in Essential Tremor: Mechanisms of Therapy Recruiting NCT02712515
7 Longitudinal Study of Neurodegenerative Disorders Recruiting NCT03333200
8 Early Diagnosis of Alzheimer-like Dementia: Benefit of MRI and PET Imaging Terminated NCT01815112 Not Applicable

Search NIH Clinical Center for Cerebral Degeneration

Genetic Tests for Cerebral Degeneration

Genetic tests related to Cerebral Degeneration:

# Genetic test Affiliating Genes
1 Cerebral Degeneration 29

Anatomical Context for Cerebral Degeneration

MalaCards organs/tissues related to Cerebral Degeneration:

41
Brain, Liver, Cortex

Publications for Cerebral Degeneration

Articles related to Cerebral Degeneration:

(show all 40)
# Title Authors Year
1
Cerebral Degeneration in Amyotrophic Lateral Sclerosis Revealed by 3-Dimensional Texture Analysis. ( 27064416 )
2016
2
Split Hand / Foot Malformation Syndrome with Cerebral Degeneration. ( 27730782 )
2016
3
Proteomic analysis of aging brain in SAMP10 mouse: a model of age-related cerebral degeneration. ( 20471994 )
2010
4
Limbic structures are prone to age-related impairments in proteasome activity and neuronal ubiquitinated inclusions in SAMP10 mouse: a model of cerebral degeneration. ( 17973906 )
2008
5
Reduced response of splenocytes after mitogen-stimulation in the prion protein (PrP) gene-deficient mouse: PrPLP/Doppel production and cerebral degeneration. ( 17498663 )
2007
6
Cerebral degeneration predicts survival in amyotrophic lateral sclerosis. ( 16835288 )
2006
7
Apical vulnerability to dendritic retraction in prefrontal neurones of ageing SAMP10 mouse: a model of cerebral degeneration. ( 16409548 )
2006
8
Detection of cerebral degeneration in amyotrophic lateral sclerosis using high-field magnetic resonance spectroscopy. ( 16908742 )
2006
9
Age-related loss of synapses in the frontal cortex of SAMP10 mouse: a model of cerebral degeneration. ( 12687639 )
2003
10
Slowly progressive pure dysgraphia with late apraxia of speech: a further variant of the focal cerebral degeneration. ( 14642538 )
2003
11
Age-related progressive neuronal DNA damage associated with cerebral degeneration in a mouse model of accelerated senescence. ( 12456731 )
2002
12
Progressive cerebral degeneration of childhood with liver disease (Alpers Huttenlocher disease) with cytochrome oxidase deficiency presenting with epilepsia partialis continua as the first clinical manifestation. ( 9561326 )
1998
13
Spino-cerebello-cerebral degeneration with amyloid plaques (Gerstmann, StrAoussler, Scheinker syndrome). ( 7151028 )
1982
14
Cerebral degeneration in Down's syndrome. ( 6459258 )
1981
15
Electroencephalographic studies on hepato-cerebral degeneration. ( 7291928 )
1981
16
Multicystic cerebral degeneration in neonatal herpes simplex virus encephalitis. ( 193394 )
1977
17
Infantile diffuse cerebral degeneration with hepatic cirrhosis. ( 1252162 )
1976
18
Distribution of cerebral degeneration in Alzheimer's disease. A clinico-pathological study. ( 828039 )
1976
19
Letter: Adult acute leukemia: cerebral degeneration. ( 4529107 )
1974
20
Norrie's disease (hereditary oculo-acoustic-cerebral degeneration). Report of a United States family. ( 4201220 )
1973
21
Cerebral degeneration with hypertrophy of the contralateral pyramid. ( 5008324 )
1972
22
The old age syndrome: subtle cerebral degeneration and bronchopneumonia. ( 5012283 )
1972
23
Norrie's disease. Congenital progressive oculo-acoustico-cerebral degeneration. ( 5000913 )
1971
24
Combined hepatic and cerebral degeneration in infancy. ( 5667851 )
1968
25
On some aspects of cerebral degeneration in later life. ( 20918952 )
1967
26
Norrie's disease. A congenital progressive oculo-acoustico-cerebral degeneration. ( 6013082 )
1966
27
Bilateral cherry-red spots without clinical cerebral degeneration. ( 5841578 )
1965
28
Diffuse cerebral degeneration in infancy (Alpers' disease). ( 13971413 )
1963
29
Progessive cerebral degeneration of infancy. ( 13792917 )
1960
30
Affective disorders arising in the senium. I. Their association with organic cerebral degeneration. ( 13243045 )
1955
31
Rapidly progressive cerebral degeneration (subacute vascular encephalopathy) with mental disorder, focal disturbances, and myoclonic epilepsy. ( 13163708 )
1954
32
Heredofamilial infantile cerebral degeneration: the relationship of systemic factors to the pathogenicity. ( 13152645 )
1954
33
Two Cases of Early Familial Maculo-cerebral Degeneration. ( 19983252 )
1923
34
Case of Cerebral Degeneration. ( 19983330 )
1923
35
Familial Cerebral Degeneration. ( 19982580 )
1922
36
Cerebral Degeneration and Epileptiform Fits, with Amaurosis, in an Only Child. ( 19979882 )
1917
37
Family Cerebral Degeneration with Macular Changes. ( 19978990 )
1915
38
Cerebral Degeneration with Changes at the Macula in four members of a family. ( 19973149 )
1908
39
Infantile Cerebral Degeneration with Symmetrical Changes at the Macula. ( 20896909 )
1897
40
Case of Grave Lesion of Both Corpora Striata: Recovery from Hemiplegia; Extensive Cerebral Degeneration; Dementia; Death Twelve Days after Rupture of Intrathoracic Aneurism. ( 20748050 )
1875

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 12.09 GFAP L1CAM NOTCH3 NOTCH4
2 11.4 NOTCH3 NOTCH4 PIK3R2
3 11.14 GFAP NOTCH3 NOTCH4
4 10.94 ABCD1 ABCD2 ABCD3 PEX5 SLC25A17

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 9.5 GFAP GJC2 PLP1
2 peroxisome GO:0005777 9.35 ABCD1 ABCD2 ABCD3 PEX5 SLC25A17
3 integral component of peroxisomal membrane GO:0005779 9.26 ABCD1 SLC25A17
4 eukaryotic translation initiation factor 2B complex GO:0005851 9.16 EIF2B2 EIF2B5
5 peroxisomal membrane GO:0005778 9.02 ABCD1 ABCD2 ABCD3 PEX5 SLC25A17

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

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 transmembrane transport GO:0055085 9.89 ABCD1 ABCD2 ABCD3 GJC2 SLC25A17
2 myelination GO:0042552 9.61 EIF2B2 EIF2B5 PLP1
3 oligodendrocyte development GO:0014003 9.51 EIF2B2 EIF2B5
4 peroxisome organization GO:0007031 9.5 ABCD1 ABCD3 PEX5
5 positive regulation of transcription of Notch receptor target GO:0007221 9.48 NOTCH3 NOTCH4
6 central nervous system myelination GO:0022010 9.46 ASPA PLP1
7 astrocyte development GO:0014002 9.43 EIF2B5 GFAP PLP1
8 regulation of developmental process GO:0050793 9.4 NOTCH3 NOTCH4
9 peroxisomal long-chain fatty acid import GO:0015910 9.37 ABCD1 ABCD3
10 axon development GO:0061564 9.33 GFAP L1CAM PLP1
11 very long-chain fatty acid catabolic process GO:0042760 9.13 ABCD1 ABCD2 ABCD3
12 fatty acid beta-oxidation GO:0006635 9.02 ABCD1 ABCD2 ABCD3 PEX5 SLC25A17

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

# Name GO ID Score Top Affiliating Genes
1 ATPase activity, coupled to transmembrane movement of substances GO:0042626 9.13 ABCD1 ABCD2 ABCD3
2 long-chain fatty acid transporter activity GO:0005324 8.8 ABCD1 ABCD2 ABCD3
3 protein binding GO:0005515 10.09 ABCD1 ABCD2 ABCD3 ASPA EIF2B2 EIF2B5

Sources for Cerebral 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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