MCID: CRB033
MIFTS: 41

Cerebral Degeneration

Categories: Neuronal diseases

Aliases & Classifications for Cerebral Degeneration

MalaCards integrated aliases for Cerebral Degeneration:

Name: Cerebral Degeneration 12 15
Degenerative Brain Disorder 74
Brain Degeneration 12

Classifications:



External Ids:

Disease Ontology 12 DOID:1443
ICD9CM 36 331.9
SNOMED-CT 69 52522001 73768007
UMLS 74 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 mitochondrial dna depletion syndrome 4a. An important gene associated with Cerebral Degeneration is NOTCH3 (Notch Receptor 3), and among its related pathways/superpathways are Translation Factors and Peroxisome. The drugs Glucagon and Triamcinolone have been mentioned in the context of this disorder. Affiliated tissues include brain, liver and cortex, and related phenotypes are no effect and behavior/neurological

Wikipedia : 77 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 53)
# Related Disease Score Top Affiliating Genes
1 leukodystrophy 31.0 ASPA EIF2B1 EIF2B2 EIF2B5 GFAP GJC2
2 mitochondrial dna depletion syndrome 4a 11.6
3 norrie disease 11.1
4 niemann-pick disease 11.0
5 brain small vessel disease 1 with or without ocular anomalies 11.0
6 brain small vessel disease 2 11.0
7 hydrocephalus 11.0
8 canavan disease 10.8
9 lipid storage disease 10.8
10 adrenomyeloneuropathy 10.3 ABCD1 SLC25A17
11 pelizaeus-merzbacher-like disease 10.3 GJC2 PLP1
12 inflammatory mfh 10.3 GFAP SERPINA3
13 leukodystrophy, hypomyelinating, 4 10.3 GJC2 PLP1
14 binswanger's disease 10.3 GFAP NOTCH3 PLP1
15 hypomyelinating leukoencephalopathy 10.3 GJC2 PLP1
16 spastic paraplegia 2, x-linked 10.3 GJC2 PLP1
17 cortical blindness 10.3 ABCD1 POLG
18 demyelinating disease 10.2 ABCD1 ABCD2 PLP1
19 adrenoleukodystrophy 10.2 ABCD1 ABCD2 ABCD3
20 aceruloplasminemia 10.2 GFAP PLA2G6 POLG
21 spinocerebellar degeneration 10.2 ABCD1 POLG
22 hereditary spastic paraplegia 10.2 GJC2 L1CAM PLA2G6 PLP1
23 leukoencephalopathy with vanishing white matter 10.2 EIF2B1 EIF2B2 EIF2B5
24 granular cell tumor 10.0 GFAP SERPINA3
25 x-linked recessive disease 10.0 ABCD1 ABCD2 ABCD3 PLP1 SLC25A17
26 amyotrophic lateral sclerosis 1 9.9
27 lateral sclerosis 9.9
28 intermittent claudication 9.8
29 fetal brain disruption sequence 9.8
30 alzheimer disease 9.6
31 pityriasis rubra pilaris 9.6
32 aging 9.6
33 acute leukemia 9.6
34 leukemia 9.6
35 liver disease 9.6
36 apraxia 9.6
37 split hand-foot malformation 9.6
38 hemiplegia 9.6
39 bronchopneumonia 9.6
40 dementia 9.6
41 epilepsy 9.6
42 early myoclonic encephalopathy 9.6
43 dysgraphia 9.6
44 herpes simplex 9.6
45 encephalitis 9.6
46 neonatal herpes 9.6
47 encephalopathy 9.6
48 machado-joseph disease 9.6
49 spinocerebellar ataxia 2 9.6
50 wilson disease 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:

27
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 no effect GR00402-S-1 9.92 ABCD1 ABCD2 ABCD3 ASAH1 ASPA DCAF8

MGI Mouse Phenotypes related to Cerebral Degeneration:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.23 ABCD1 ABCD2 ASAH1 ASPA EIF2B5 GFAP
2 hematopoietic system MP:0005397 10.18 ABCD1 ABCD2 ASAH1 ASPA EIF2B2 EIF2B5
3 cellular MP:0005384 10.13 ABCD3 ASAH1 GFAP GJC2 L1CAM NOTCH3
4 immune system MP:0005387 10.11 ABCD1 ABCD2 ASAH1 EIF2B5 GFAP GJC2
5 mortality/aging MP:0010768 10.1 ABCD1 ASAH1 ASPA EIF2B5 GFAP GJC2
6 nervous system MP:0003631 10.1 ABCD1 ABCD2 ASAH1 ASPA EIF2B5 GFAP
7 reproductive system MP:0005389 9.76 ABCD1 ASAH1 L1CAM NOTCH3 PLA2G6 PLP1
8 skeleton MP:0005390 9.56 ASAH1 ASPA L1CAM NOTCH3 PLA2G6 PLP1
9 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 13)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Glucagon Approved Not Applicable 16941-32-5
2
Triamcinolone Approved, Vet_approved Not Applicable 124-94-7 31307
3 Nutrients Not Applicable
4 Incretins Not Applicable
5 Hormones Not Applicable
6 Gastrointestinal Agents Not Applicable
7 Glucagon-Like Peptide 1 Not Applicable
8 Hormones, Hormone Substitutes, and Hormone Antagonists Not Applicable
9 Hormone Antagonists Not Applicable
10 Triamcinolone hexacetonide Not Applicable
11 Triamcinolone diacetate Not Applicable
12 triamcinolone acetonide Not Applicable
13 Fluorodeoxyglucose F18 Not Applicable

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 MRI Biomarkers in ALS Completed NCT02405182
2 To Determine the Effect of GLP1 on Cognitive Function, Brain Glucose Metabolism and Neuroplasticity. Completed 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

Anatomical Context for Cerebral Degeneration

MalaCards organs/tissues related to Cerebral Degeneration:

42
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
Apical vulnerability to dendritic retraction in prefrontal neurones of ageing SAMP10 mouse: a model of cerebral degeneration. ( 16409548 )
2006
9
Cerebral degeneration predicts survival in amyotrophic lateral sclerosis. ( 16835288 )
2006
10
Detection of cerebral degeneration in amyotrophic lateral sclerosis using high-field magnetic resonance spectroscopy. ( 16908742 )
2006
11
Slowly progressive pure dysgraphia with late apraxia of speech: a further variant of the focal cerebral degeneration. ( 14642538 )
2003
12
Age-related loss of synapses in the frontal cortex of SAMP10 mouse: a model of cerebral degeneration. ( 12687639 )
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, Sträussler, 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
Distribution of cerebral degeneration in Alzheimer's disease. A clinico-pathological study. ( 828039 )
1976
20
Infantile diffuse cerebral degeneration with hepatic cirrhosis. ( 1252162 )
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
The old age syndrome: subtle cerebral degeneration and bronchopneumonia. ( 5012283 )
1972
24
Cerebral degeneration with hypertrophy of the contralateral pyramid. ( 5008324 )
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
Heredofamilial infantile cerebral degeneration: the relationship of systemic factors to the pathogenicity. ( 13152645 )
1954
34
Rapidly progressive cerebral degeneration (subacute vascular encephalopathy) with mental disorder, focal disturbances, and myoclonic epilepsy. ( 13163708 )
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
Show member pathways
10.92 EIF2B1 EIF2B2 EIF2B5
2 10.84 ABCD1 ABCD2 ABCD3 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 peroxisome GO:0005777 9.46 ABCD1 ABCD2 ABCD3 SLC25A17
2 myelin sheath GO:0043209 9.32 GJC2 PLP1
3 integral component of peroxisomal membrane GO:0005779 9.26 ABCD1 SLC25A17
4 peroxisomal membrane GO:0005778 9.26 ABCD1 ABCD2 ABCD3 SLC25A17
5 eukaryotic translation initiation factor 2B complex GO:0005851 8.8 EIF2B1 EIF2B2 EIF2B5

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

(show all 22)
# Name GO ID Score Top Affiliating Genes
1 transmembrane transport GO:0055085 9.97 ABCD1 ABCD2 ABCD3 GJC2 SLC25A17
2 translational initiation GO:0006413 9.78 EIF2B1 EIF2B2 EIF2B5
3 T cell receptor signaling pathway GO:0050852 9.77 EIF2B1 EIF2B2 EIF2B5
4 response to glucose GO:0009749 9.69 EIF2B1 EIF2B2 EIF2B5
5 response to peptide hormone GO:0043434 9.67 EIF2B1 EIF2B2 EIF2B5
6 myelination GO:0042552 9.65 EIF2B2 EIF2B5 PLP1
7 response to heat GO:0009408 9.63 EIF2B1 EIF2B2 EIF2B5
8 peroxisome organization GO:0007031 9.6 ABCD1 ABCD3
9 negative regulation of cytokine production involved in inflammatory response GO:1900016 9.58 ABCD1 ABCD2
10 oligodendrocyte development GO:0014003 9.58 EIF2B1 EIF2B2 EIF2B5
11 cellular metabolic process GO:0044237 9.57 EIF2B1 EIF2B2
12 negative regulation of reactive oxygen species biosynthetic process GO:1903427 9.56 ABCD1 ABCD2
13 central nervous system myelination GO:0022010 9.55 ASPA PLP1
14 neuron projection maintenance GO:1990535 9.54 ABCD1 ABCD2
15 positive regulation of fatty acid beta-oxidation GO:0032000 9.52 ABCD1 ABCD2
16 myelin maintenance GO:0043217 9.43 ABCD1 ABCD2
17 astrocyte development GO:0014002 9.43 EIF2B5 GFAP PLP1
18 peroxisomal long-chain fatty acid import GO:0015910 9.37 ABCD1 ABCD3
19 axon development GO:0061564 9.33 GFAP L1CAM PLP1
20 positive regulation of unsaturated fatty acid biosynthetic process GO:2001280 9.32 ABCD1 ABCD2
21 fatty acid beta-oxidation GO:0006635 9.26 ABCD1 ABCD2 ABCD3 SLC25A17
22 very long-chain fatty acid catabolic process GO:0042760 8.8 ABCD1 ABCD2 ABCD3

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

# Name GO ID Score Top Affiliating Genes
1 guanyl-nucleotide exchange factor activity GO:0005085 9.5 EIF2B1 EIF2B2 EIF2B5
2 translation initiation factor activity GO:0003743 9.33 EIF2B1 EIF2B2 EIF2B5
3 ATPase activity, coupled to transmembrane movement of substances GO:0042626 9.13 ABCD1 ABCD2 ABCD3
4 long-chain fatty acid transporter activity GO:0005324 8.62 ABCD1 ABCD3
5 protein binding GO:0005515 10.16 ABCD1 ABCD2 ABCD3 ASPA DCAF8 EIF2B1

Sources for Cerebral 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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