MCID: CRB033
MIFTS: 43

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:



External Ids:

Disease Ontology 12 DOID:1443
ICD9CM 35 331.9
SNOMED-CT 68 52522001 73768007
UMLS 73 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 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

Wikipedia : 76 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 all 49)
# Related Disease Score Top Affiliating Genes
1 hydrocephalus 31.5 GFAP L1CAM PIK3R2 ZIC3
2 leukodystrophy 31.1 ASPA EIF2B2 EIF2B5 FA2H GFAP GJC2
3 mitochondrial dna depletion syndrome 4a 11.6
4 norrie disease 11.1
5 niemann-pick disease 10.9
6 porencephaly 2 10.9
7 canavan disease 10.8
8 lipid storage disease 10.8
9 pseudobulbar palsy 10.3 NOTCH3 NOTCH4
10 pelizaeus-merzbacher-like disease 10.2 GJC2 PLP1
11 adrenomyeloneuropathy 10.2 ABCD1 SLC25A17
12 leukodystrophy, hypomyelinating, 4 10.2 GJC2 PLP1
13 binswanger's disease 10.2 GFAP NOTCH3 PLP1
14 hypomyelinating leukoencephalopathy 10.2 GJC2 PLP1
15 chorioretinal scar 10.2 NOTCH3 NOTCH4
16 spastic paraplegia 2, x-linked 10.2 GJC2 PLP1
17 leukodystrophy, hypomyelinating, 2 10.2 FA2H GJC2 PLP1
18 demyelinating disease 10.1 ABCD1 ABCD2 PLP1
19 cortical blindness 10.1 ABCD1 POLG
20 aceruloplasminemia 10.1 FA2H GFAP POLG
21 hereditary spastic paraplegia 10.1 FA2H GJC2 L1CAM PLP1
22 adrenoleukodystrophy 10.1 ABCD1 ABCD2 ABCD3 PEX5
23 spinocerebellar degeneration 10.1 ABCD1 POLG
24 amyotrophic lateral sclerosis 1 9.9
25 lateral sclerosis 9.9
26 x-linked recessive disease 9.9 ABCD1 ABCD2 ABCD3 PEX5 PLP1 SLC25A17
27 fetal brain disruption sequence 9.8
28 alzheimer disease 9.6
29 aging 9.6
30 acute leukemia 9.6
31 intermittent claudication 9.6
32 leukemia 9.6
33 liver disease 9.6
34 apraxia 9.6
35 split hand-foot malformation 9.6
36 hemiplegia 9.6
37 bronchopneumonia 9.6
38 dementia 9.6
39 epilepsy 9.6
40 dysgraphia 9.6
41 herpes simplex 9.6
42 encephalitis 9.6
43 neonatal herpes 9.6
44 encephalopathy 9.6
45 machado-joseph disease 9.6
46 spinocerebellar ataxia 2 9.6
47 wilson disease 9.6
48 microhydranencephaly 9.6
49 autosomal dominant cerebellar ataxia 9.6

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 ASAH1 EIF2B5 FA2H GFAP
8 Decreased viability GR00402-S-2 10.13 ABCD1 ABCD2 ABCD3 ASAH1 ASPA EIF2B2
9 no effect GR00402-S-1 9.62 ABCD1 ABCD2 ABCD3 ASAH1 ASPA EIF2B2

MGI Mouse Phenotypes related to Cerebral Degeneration:

46 (show all 12)
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.32 ABCD1 ABCD2 ASAH1 ASPA EIF2B5 FA2H
2 homeostasis/metabolism MP:0005376 10.28 ABCD1 ABCD2 ABCD3 ASAH1 ASPA EIF2B5
3 growth/size/body region MP:0005378 10.26 ASAH1 ASPA EIF2B5 GFAP L1CAM NOTCH3
4 hematopoietic system MP:0005397 10.23 ABCD1 ABCD2 ASAH1 ASPA EIF2B2 EIF2B5
5 cellular MP:0005384 10.19 ABCD3 ASAH1 GFAP GJC2 L1CAM NOTCH3
6 mortality/aging MP:0010768 10.18 ABCD1 ASAH1 ASPA EIF2B5 GFAP GJC2
7 nervous system MP:0003631 10.16 ABCD1 ABCD2 ASAH1 ASPA EIF2B5 FA2H
8 immune system MP:0005387 10.14 ABCD1 ABCD2 ASAH1 EIF2B5 GFAP GJC2
9 muscle MP:0005369 9.87 ASPA GFAP NOTCH3 PEX5 PIK3R2 PLP1
10 reproductive system MP:0005389 9.76 ABCD1 ASAH1 L1CAM NOTCH3 PEX5 PLP1
11 skeleton MP:0005390 9.56 ASAH1 ASPA L1CAM NOTCH3 PEX5 PLP1
12 vision/eye MP:0005391 9.36 ABCD1 ASPA EIF2B5 FA2H GFAP GJC2

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 Incretins Not Applicable
5 Hormone Antagonists Not Applicable
6 Glucagon-Like Peptide 1 Not Applicable
7 Hormones Not Applicable
8 Hormones, Hormone Substitutes, and Hormone Antagonists Not Applicable
9 triamcinolone acetonide Not Applicable
10 Triamcinolone hexacetonide Not Applicable
11 Triamcinolone diacetate Not Applicable
12 Fluorodeoxyglucose F18 Not Applicable

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 To Determine the Effect of GLP1 on Cognitive Function, Brain Glucose Metabolism and Neuroplasticity. Completed NCT03414333 Not Applicable GLP-1
2 MRI Biomarkers in ALS Recruiting NCT02405182
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, Fetal Brain

Publications for Cerebral Degeneration

Articles related to Cerebral Degeneration:

(show all 42)
# Title Authors Year
1
Texture Analysis to Detect Cerebral Degeneration in Amyotrophic Lateral Sclerosis. ( 30234470 )
2018
2
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'. ( 28332229 )
2017
3
Cerebral Degeneration in Amyotrophic Lateral Sclerosis Revealed by 3-Dimensional Texture Analysis. ( 27064416 )
2016
4
Split Hand / Foot Malformation Syndrome with Cerebral Degeneration. ( 27730782 )
2016
5
Proteomic analysis of aging brain in SAMP10 mouse: a model of age-related cerebral degeneration. ( 20471994 )
2010
6
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
7
Reduced response of splenocytes after mitogen-stimulation in the prion protein (PrP) gene-deficient mouse: PrPLP/Doppel production and cerebral degeneration. ( 17498663 )
2007
8
Cerebral degeneration predicts survival in amyotrophic lateral sclerosis. ( 16835288 )
2006
9
Apical vulnerability to dendritic retraction in prefrontal neurones of ageing SAMP10 mouse: a model of cerebral degeneration. ( 16409548 )
2006
10
Detection of cerebral degeneration in amyotrophic lateral sclerosis using high-field magnetic resonance spectroscopy. ( 16908742 )
2006
11
Age-related loss of synapses in the frontal cortex of SAMP10 mouse: a model of cerebral degeneration. ( 12687639 )
2003
12
Slowly progressive pure dysgraphia with late apraxia of speech: a further variant of the focal cerebral degeneration. ( 14642538 )
2003
13
Age-related progressive neuronal DNA damage associated with cerebral degeneration in a mouse model of accelerated senescence. ( 12456731 )
2002
14
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
15
Spino-cerebello-cerebral degeneration with amyloid plaques (Gerstmann, StrAoussler, Scheinker syndrome). ( 7151028 )
1982
16
Cerebral degeneration in Down's syndrome. ( 6459258 )
1981
17
Electroencephalographic studies on hepato-cerebral degeneration. ( 7291928 )
1981
18
Multicystic cerebral degeneration in neonatal herpes simplex virus encephalitis. ( 193394 )
1977
19
Infantile diffuse cerebral degeneration with hepatic cirrhosis. ( 1252162 )
1976
20
Distribution of cerebral degeneration in Alzheimer's disease. A clinico-pathological study. ( 828039 )
1976
21
Letter: Adult acute leukemia: cerebral degeneration. ( 4529107 )
1974
22
Norrie's disease (hereditary oculo-acoustic-cerebral degeneration). Report of a United States family. ( 4201220 )
1973
23
Cerebral degeneration with hypertrophy of the contralateral pyramid. ( 5008324 )
1972
24
The old age syndrome: subtle cerebral degeneration and bronchopneumonia. ( 5012283 )
1972
25
Norrie's disease. Congenital progressive oculo-acoustico-cerebral degeneration. ( 5000913 )
1971
26
Combined hepatic and cerebral degeneration in infancy. ( 5667851 )
1968
27
On some aspects of cerebral degeneration in later life. ( 20918952 )
1967
28
Norrie's disease. A congenital progressive oculo-acoustico-cerebral degeneration. ( 6013082 )
1966
29
Bilateral cherry-red spots without clinical cerebral degeneration. ( 5841578 )
1965
30
Diffuse cerebral degeneration in infancy (Alpers' disease). ( 13971413 )
1963
31
Progessive cerebral degeneration of infancy. ( 13792917 )
1960
32
Affective disorders arising in the senium. I. Their association with organic cerebral degeneration. ( 13243045 )
1955
33
Rapidly progressive cerebral degeneration (subacute vascular encephalopathy) with mental disorder, focal disturbances, and myoclonic epilepsy. ( 13163708 )
1954
34
Heredofamilial infantile cerebral degeneration: the relationship of systemic factors to the pathogenicity. ( 13152645 )
1954
35
Two Cases of Early Familial Maculo-cerebral Degeneration. ( 19983252 )
1923
36
Case of Cerebral Degeneration. ( 19983330 )
1923
37
Familial Cerebral Degeneration. ( 19982580 )
1922
38
Cerebral Degeneration and Epileptiform Fits, with Amaurosis, in an Only Child. ( 19979882 )
1917
39
Family Cerebral Degeneration with Macular Changes. ( 19978990 )
1915
40
Cerebral Degeneration with Changes at the Macula in four members of a family. ( 19973149 )
1908
41
Infantile Cerebral Degeneration with Symmetrical Changes at the Macula. ( 20896909 )
1897
42
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
5 10.75 NOTCH3 NOTCH4

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 peroxisome GO:0005777 9.35 ABCD1 ABCD2 ABCD3 PEX5 SLC25A17
2 integral component of peroxisomal membrane GO:0005779 9.26 ABCD1 SLC25A17
3 eukaryotic translation initiation factor 2B complex GO:0005851 9.16 EIF2B2 EIF2B5
4 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 19)
# Name GO ID Score Top Affiliating Genes
1 transmembrane transport GO:0055085 9.95 ABCD1 ABCD2 ABCD3 GJC2 SLC25A17
2 T cell receptor signaling pathway GO:0050852 9.75 EIF2B2 EIF2B5 PIK3R2
3 myelination GO:0042552 9.65 EIF2B2 EIF2B5 PLP1
4 oligodendrocyte development GO:0014003 9.58 EIF2B2 EIF2B5
5 negative regulation of cytokine production involved in inflammatory response GO:1900016 9.57 ABCD1 ABCD2
6 positive regulation of transcription of Notch receptor target GO:0007221 9.56 NOTCH3 NOTCH4
7 negative regulation of reactive oxygen species biosynthetic process GO:1903427 9.54 ABCD1 ABCD2
8 central nervous system myelination GO:0022010 9.52 ASPA PLP1
9 neuron projection maintenance GO:1990535 9.51 ABCD1 ABCD2
10 peroxisome organization GO:0007031 9.5 ABCD1 ABCD3 PEX5
11 positive regulation of fatty acid beta-oxidation GO:0032000 9.49 ABCD1 ABCD2
12 myelin maintenance GO:0043217 9.46 ABCD1 ABCD2
13 regulation of developmental process GO:0050793 9.43 NOTCH3 NOTCH4
14 astrocyte development GO:0014002 9.43 EIF2B5 GFAP PLP1
15 peroxisomal long-chain fatty acid import GO:0015910 9.4 ABCD1 ABCD3
16 positive regulation of unsaturated fatty acid biosynthetic process GO:2001280 9.37 ABCD1 ABCD2
17 axon development GO:0061564 9.33 GFAP L1CAM PLP1
18 very long-chain fatty acid catabolic process GO:0042760 9.13 ABCD1 ABCD2 ABCD3
19 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 10.06 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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