MCID: SMN008
MIFTS: 50

Semantic Dementia malady

Rare diseases, Neuronal diseases, Mental diseases categories

Summaries for Semantic Dementia

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Sources:
45NINDS, 66Wikipedia, 34MalaCards
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NINDS:45 Frontotemporal dementia (FTD) describes a clinical syndrome associated with shrinking of the frontal and temporal anterior lobes of the brain. Originally known as Pick?s disease, the name and classification of FTD has been a topic of discussion for over a century.

MalaCards: Semantic Dementia, also known as semantic primary progressive aphasia, is related to dementia and frontotemporal dementia. An important gene associated with Semantic Dementia is GRN (granulin), and among its related pathways are Budding and maturation of HIV virion and Fanconi anemia pathway. The compounds tacrine and donepezil have been mentioned in the context of this disorder. Affiliated tissues include brain, temporal lobe and testes, and related mouse phenotypes are other and no phenotypic analysis.

Wikipedia:66 Semantic dementia (SD) is a progressive neurodegenerative disorder characterized by loss of semantic... more...

Aliases & Classifications for Semantic Dementia

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Sources:
44NIH Rare Diseases, 45NINDS, 46Novoseek, 50Orphanet, 63UMLS, 64UMLS via Orphanet, 60SNOMED-CT via Orphanet, 27ICD10 via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Neuronal diseases, Mental diseases


Characteristics (Orphanet epidemiological data):

50
semantic dementia:
Inheritance: Multigenic/multifactorial,Sporadic; Age of onset: Adulthood


Aliases & Descriptions:

semantic dementia 44 45 46 50 63
semantic primary progressive aphasia 44 50
semantic variant ppa 44 50
primary progressive aphasia 63
dementia - semantic 45


External Ids:

UMLS via Orphanet64 C0338462
SNOMED-CT via Orphanet60 230288001
ICD10 via Orphanet27 G31.0

Related Diseases for Semantic Dementia

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Sources:
18GeneCards, 19GeneDecks
See all sources

Diseases related to Semantic Dementia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show all 45)
idRelated DiseaseScoreTop Affiliating Genes
1dementia31.7APOE, VCP, TREM2, TARDBP, GRN, MAPT
2frontotemporal dementia31.3PSEN1, MAPT
3aphasia31.2C9orf72, MAPT, GRN, TARDBP
4alzheimer's disease31.1APOE, VCP, PSEN1, RPS27A, MAPT, GRN
5primary progressive aphasia30.9GRN, MAPT, RPS27A
6memory impairment30.6GRN, MAPT, PSEN1, APOE
7motor neuron disease30.3VCP, RPS27A, MAPT, GRN, TARDBP
8apraxia30.3GRN, MAPT
9pick's disease30.3PSEN1, MAPT
10tauopathy30.2PSEN1, RPS27A, MAPT, GRN
11dyslexia10.6
12neuronitis10.4
13cerebritis10.4
14corticobasal degeneration10.4
15alexia10.3
16dysgraphia10.1
17prosopagnosia10.1
18hyperacusis10.1
19progressive non-fluent aphasia10.1
20frontotemporal lobar degeneration with ubiquitin-positive inclusions10.1GRN
21neuronal intranuclear inclusion disease10.1RPS27A
22lateral sclerosis10.1TARDBP
23inclusion body myositis10.1MAPT, RPS27A
24spinocerebellar ataxia10.1MAPT, RPS27A
25lewy body dementia10.0APOE, MAPT
26movement disease10.0MAPT, RPS27A
27prion disease10.0MAPT, PSEN1
28progressive supranuclear palsy10.0APOE, RPS27A, MAPT
29creutzfeldt-jakob disease10.0APOE, RPS27A, MAPT
30multiple system atrophy10.0RPS27A, MAPT
31parkinson's disease10.0MAPT, RPS27A, APOE
32myositis10.0PSEN1, RPS27A, MAPT
33huntington's disease10.0PSEN1, RPS27A, MAPT
34teratocarcinoma10.0MAPT, PSEN1
35hemorrhage, intracerebral10.0APOE, PSEN1, RPS27A
36alzheimer disease type 210.0APOE, PSEN1, MAPT
37cerebral amyloid angiopathy10.0APOE, PSEN1, MAPT
38spinocerebellar ataxia type 310.0VCP, RPS27A
39down syndrome10.0MAPT, PSEN1, APOE
40brain disease10.0PSEN1, MAPT
41vascular dementia10.0APOE, PSEN1, RPS27A, MAPT
42amyloid tumor10.0APOE, PSEN1, RPS27A, MAPT
43amyloidosis10.0APOE, PSEN1, RPS27A, MAPT
44cerebrovascular disease10.0MAPT, PSEN1, APOE
45amyotrophic lateral sclerosis9.8TARDBP, GRN, MAPT, RPS27A, PSEN1, CHMP2B

Graphical network of the top 20 diseases related to Semantic Dementia:



Diseases related to semantic dementia

Symptoms for Semantic Dementia

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Drugs & Therapeutics for Semantic Dementia

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Semantic Dementia

Drug clinical trials:

Search ClinicalTrials for Semantic Dementia

Search NIH Clinical Center for Semantic Dementia

Search CenterWatch for Semantic Dementia

Genetic Tests for Semantic Dementia

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Anatomical Context for Semantic Dementia

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Sources:
34MalaCards
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MalaCards organs/tissues related to Semantic Dementia:

34
Brain, Temporal lobe, Testes, Cortex

Animal Models for Semantic Dementia or affiliated genes

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Sources:
38MGI
See all sources

MGI Mouse Phenotypes related to Semantic Dementia:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053959.0MAPT, PSEN1, APOE
2MP:00030128.2APOE, C9orf72, MAPT, GRN, TARDBP
3MP:00053698.1APOE, VCP, PSEN1, MAPT, TARDBP
4MP:00053977.8APOE, VCP, PSEN1, MAPT, GRN, TARDBP
5MP:00053867.7APOE, VCP, PSEN1, MAPT, GRN, TARDBP
6MP:00053847.7APOE, VCP, PSEN1, MAPT, GRN, TARDBP
7MP:00107687.7VCP, PSEN1, MAPT, GRN, TARDBP, APOE
8MP:00036317.6APOE, VCP, PSEN1, MAPT, GRN, TARDBP
9MP:00053877.5TREM2, TARDBP, GRN, MAPT, PSEN1, VCP

Publications for Semantic Dementia

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Sources:
53PubMed
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Articles related to Semantic Dementia:

(show top 50)    (show all 286)
idTitleAuthorsYear
1
Cerebellar hyperperfusion in semantic dementia. (23414345)
2014
2
Machine learning approaches to diagnosis and laterality effects in semantic dementia discourse. (23876449)
2013
3
Phonology without semantics? Good enough for verbal short-term memory. Evidence from a patient with semantic dementia. (22664140)
2013
4
Ever decreasing circles: Speech production in semantic dementia. (23561299)
2013
5
Demonstrating the qualitative differences between semantic aphasia and semantic dementia: a novel exploration of nonverbal semantic processing. (22713375)
2013
6
Late-onset OCD as presenting manifestation of semantic dementia. (21908052)
2012
7
Considering the role of semantic memory in episodic future thinking: evidence from semantic dementia. (22614246)
2012
8
Semantic dementia without surface dyslexia in Spanish: unimpaired reading with impaired semantics. (22713391)
2012
9
Reverse concreteness effects are not a typical feature of semantic dementia: evidence for the hub-and-spoke model of conceptual representation. (21285258)
2011
10
Clinical profiles of late-onset semantic dementia, compared with early-onset semantic dementia and late-onset Alzheimer's disease. (21447109)
2011
11
Verb production during action naming in semantic dementia. (21237467)
2011
12
Anomia as a marker of distinct semantic memory impairments in Alzheimer's disease and semantic dementia. (21443339)
2011
13
Semantic dementia with lower motor neuron disease showing FTLD-TDP type 3 pathology (sensu Mackenzie). (21029204)
2011
14
Visual search patterns in semantic dementia show paradoxical facilitation of binding processes. (21215762)
2011
15
Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases. (19805492)
2010
16
Flavour processing in semantic dementia. (19656505)
2010
17
Personal experience and arithmetic meaning in semantic dementia. (19766666)
2010
18
Language networks in semantic dementia. (19759202)
2010
19
Abnormal discourse in semantic dementia: a data-driven approach. (20614366)
2010
20
Atrophy patterns in Alzheimer's disease and semantic dementia: a comparison of FreeSurfer and manual volumetric measurements. (19874902)
2010
21
'The quicksand of forgetfulness': semantic dementia in One hundred years of solitude. (19447824)
2009
22
Jigsaws-a preserved ability in semantic dementia. (19014960)
2009
23
Effects of semantic impairment on language processing in semantic dementia. (18348090)
2008
24
Arithmetic knowledge in semantic dementia: is it invariably preserved? (18586284)
2008
25
What does a patient with semantic dementia remember in verbal short-term memory? Order and sound but not words. (18416485)
2007
26
'Non-semantic' aspects of language in semantic dementia: as normal as they're said to be? (16517512)
2006
27
How Berlusconi keeps his face: a neuropsychological study in a case of semantic dementia. (16771038)
2006
28
Semantic dementia and fluent primary progressive aphasia: two sides of the same coin? (17071925)
2006
29
Autobiographical memory in semantic dementia: implication for theories of limbic-neocortical interaction in remote memory. (16765999)
2006
30
Semantic dementia, a many-worded disorder]. (17133330)
2006
31
The understanding of quantifiers in semantic dementia: a single-case study. (16801149)
2006
32
Jargon dyslexia in an individual with semantic dementia: further evidence for task-specificity in phonological output. (16251136)
2005
33
Frontotemporal dementia, semantic dementia, and Alzheimer's disease: the contribution of standard neuropsychological tests to differential diagnosis. (15681627)
2005
34
A semantic contribution to nonword recall? Evidence for intact phonological processes in semantic dementia. (21038246)
2005
35
The role of the temporal lobe semantic system in number knowledge: evidence from late-stage semantic dementia. (15716160)
2005
36
Semantic and syntactic aspects of the mass/count distinction: a case study of semantic dementia. (15780454)
2005
37
The contribution of autobiographical significance to semantic memory: evidence from Alzheimer's disease, semantic dementia, and amnesia. (14615074)
2004
38
Knowledge of famous faces and names in semantic dementia. (14985259)
2004
39
Semantic dementia in multilingual patients. (15377748)
2004
40
A duck with four legs: Investigating the structure of conceptual knowledge using picture drawing in semantic dementia. (20957563)
2003
41
Left/right asymmetry of atrophy in semantic dementia: behavioral-cognitive implications. (14610120)
2003
42
Memory consolidation and the hippocampus: further evidence from studies of autobiographical memory in semantic dementia and frontal variant frontotemporal dementia. (11792404)
2002
43
The influence of personal familiarity and context on object use in semantic dementia. (11997491)
2002
44
Differing patterns of temporal atrophy in Alzheimer's disease and semantic dementia. (11468305)
2001
45
Semantic dementia with ubiquitin-positive tau-negative inclusion bodies. (10648435)
2000
46
Combined semantic dementia and apraxia in a patient with frontotemporal lobar degeneration. (11090722)
2000
47
The rise and fall of frequency and imageability: noun and verb production in semantic dementia. (10872636)
2000
48
What does semantic dementia reveal about the functional role of the perirhinal cortex? (10377536)
1999
49
Differentiating the roles of the hippocampal complex and the neocortex in long-term memory storage: evidence from the study of semantic dementia and Alzheimer's disease. (9055272)
1997
50
Nonfluent progressive aphasia and semantic dementia: a comparative neuropsychological study. (9375155)
1996

Variations for Semantic Dementia

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Sources:
1 National Center for Biotechnology Information (Clinvar)
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Clinvar genetic disease variations for Semantic Dementia:

1
id Gene Name Type Significance SNP ID Assembly Location
1GRNNM_002087.2(GRN): c.813_816delCACT (p.Thr272Serfs)deletionPathogenicrs63749877GRCh37Chr 17, 42428509: 42428512

Expression for genes affiliated with Semantic Dementia

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Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Semantic Dementia

Search GEO for disease gene expression data for Semantic Dementia.

Pathways for genes affiliated with Semantic Dementia

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Sources:
51PathCards, 56Reactome, 31KEGG, 39NCBI BioSystems Database, 54QIAGEN, 5Cell Signaling Technology
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Pathways related to Semantic Dementia according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.4RPS27A, CHMP2B
2
Show member pathways
9.4RPS27A, VCP
3
Show member pathways
Alzheimers Disease39
9.3APOE, PSEN1, MAPT
49.3MAPT, PSEN1, APOE
58.4TARDBP, MAPT, PSEN1, APOE

Compounds for genes affiliated with Semantic Dementia

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Sources:
46Novoseek, 12DrugBank, 52PharmGKB, 25HMDB, 30IUPHAR, 3BitterDB, 62Tocris Bioscience
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Compounds related to Semantic Dementia according to GeneCards/GeneDecks:

(show all 43)
idCompoundScoreTop Affiliating Genes
1tacrine46 1211.0MAPT, APOE
2donepezil46 52 25 1213.0APOE, MAPT
3galantamine46 52 1212.0APOE, MAPT
4guanidine46 25 1211.9MAPT, APOE
5fluorochrome469.9MAPT, RPS27A
6cysteamine46 25 1211.7APOE, RPS27A
7spec-t469.7APOE, PSEN1, MAPT
8vitamin b12469.7MAPT, PSEN1, APOE
9kainate46 3010.7APOE, PSEN1, MAPT
10folate469.6MAPT, PSEN1, APOE
11glycerol46 25 1211.5MAPT, RPS27A, PSEN1
12streptozotocin469.5APOE, RPS27A
13thioflavine s469.4MAPT, RPS27A, APOE
14formate469.4MAPT, RPS27A, APOE
15chloroquine46 3 52 30 1213.4APOE, RPS27A, MAPT
16sodium dodecylsulfate469.4MAPT, RPS27A, APOE
17glycogen46 2510.4MAPT, RPS27A, PSEN1
18biotin46 25 1211.3RPS27A, PSEN1, APOE
19tamoxifen46 52 30 1212.3APOE, RPS27A, GRN
20n acetylcysteine469.3MAPT, RPS27A, APOE
21acetylcholine46 52 30 25 1213.2MAPT, RPS27A, APOE
22proline469.2MAPT, RPS27A, PSEN1
23actinomycin d469.2MAPT, RPS27A, APOE
24paraffin469.1MAPT, RPS27A, APOE
254-hydroxynonenal46 2510.1MAPT, RPS27A, PSEN1, APOE
26lactacystin469.1APOE, PSEN1, RPS27A, MAPT
27valine469.1APOE, PSEN1, RPS27A, MAPT
28nmda46 3010.1APOE, PSEN1, RPS27A, MAPT
29guanidine hydrochloride469.0RPS27A, APOE
30aspartate469.0APOE, PSEN1, RPS27A, MAPT
31threonine469.0MAPT, RPS27A, PSEN1, VCP
32cycloheximide469.0MAPT, RPS27A, PSEN1, APOE
33cyclosporin a46 30 6211.0APOE, RPS27A, MAPT
34h2o2469.0MAPT, RPS27A, PSEN1, APOE
35arginine469.0APOE, PSEN1, RPS27A, MAPT
36glutamate468.9APOE, PSEN1, RPS27A, MAPT
37estrogen468.9APOE, RPS27A, MAPT, GRN
38alanine468.9APOE, PSEN1, RPS27A, MAPT
39dopamine46 30 25 1211.9MAPT, RPS27A, APOE
40superoxide46 259.8TARDBP, PSEN1, VCP
41cysteine468.7GRN, MAPT, RPS27A, PSEN1, APOE
42glutamine468.6MAPT, RPS27A, PSEN1, VCP, APOE
43serine468.5MAPT, RPS27A, PSEN1, VCP, APOE

GO Terms for genes affiliated with Semantic Dementia

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Sources:
17Gene Ontology
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Biological processes related to Semantic Dementia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of axon extensionGO:0457739.6APOE, MAPT
2endosomal transportGO:0161979.4RPS27A, CHMP2B
3Notch receptor processingGO:0072209.4RPS27A, PSEN1
4positive regulation of proteasomal ubiquitin-dependent protein catabolic processGO:0324369.3PSEN1, VCP
5cell deathGO:0082198.0TARDBP, GRN, CHMP2B, C9orf72, APOE

Molecular functions related to Semantic Dementia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1lipoprotein particle bindingGO:0718139.5MAPT, APOE
2poly(A) RNA bindingGO:0448228.0TARDBP, GRN, RPS27A, VCP
3protein bindingGO:0055157.2C9orf72, VCP, PSEN1, MAPT, GRN, TARDBP

Products for genes affiliated with Semantic Dementia

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  • Antibodies
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Sources for Semantic Dementia

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet