MCID: SMN008
MIFTS: 50

Semantic Dementia malady

Rare diseases, Neuronal diseases, Mental diseases categories
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Summaries for Semantic Dementia

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Sources:
44NINDS, 65Wikipedia, 33MalaCards
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NINDS:44 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:65 Semantic dementia (SD) is a progressive neurodegenerative disorder characterized by loss of semantic... more...

Aliases & Classifications for Semantic Dementia

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Sources:
43NIH Rare Diseases, 44NINDS, 45Novoseek, 49Orphanet, 62UMLS, 63UMLS via Orphanet, 59SNOMED-CT via Orphanet, 26ICD10 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):

49
semantic dementia:
Inheritance: Multigenic/multifactorial,Sporadic; Age of onset: Adulthood


Aliases & Descriptions:

semantic dementia 43 44 45 49 62
semantic primary progressive aphasia 43 49
semantic variant ppa 43 49
primary progressive aphasia 62
dementia - semantic 44


External Ids:

UMLS via Orphanet63 C0338462
SNOMED-CT via Orphanet59 230288001
ICD10 via Orphanet26 G31.0

Related Diseases for Semantic Dementia

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Sources:
17GeneCards, 18GeneDecks
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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:
42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Semantic Dementia

Search NIH Clinical Center for Semantic Dementia

Genetic Tests for Semantic Dementia

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Anatomical Context for Semantic Dementia

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Sources:
33MalaCards
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MalaCards organs/tissues related to Semantic Dementia:

33
Brain, Temporal lobe, Testes, Cortex

Animal Models for Semantic Dementia or affiliated genes

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Sources:
37MGI
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MGI Mouse Phenotypes related to Semantic Dementia:

37
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:
52PubMed
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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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2BioGPS, 15Gene 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:
50PathCards, 55Reactome, 30KEGG, 38NCBI BioSystems Database, 53QIAGEN, 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 Disease38
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:
45Novoseek, 11DrugBank, 51PharmGKB, 24HMDB, 29IUPHAR, 3BitterDB, 61Tocris Bioscience
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Compounds related to Semantic Dementia according to GeneCards/GeneDecks:

(show all 43)
idCompoundScoreTop Affiliating Genes
1tacrine45 1111.0MAPT, APOE
2donepezil45 51 24 1113.0APOE, MAPT
3galantamine45 51 1112.0APOE, MAPT
4guanidine45 24 1111.9MAPT, APOE
5fluorochrome459.9MAPT, RPS27A
6cysteamine45 24 1111.7APOE, RPS27A
7spec-t459.7APOE, PSEN1, MAPT
8vitamin b12459.7MAPT, PSEN1, APOE
9kainate45 2910.7APOE, PSEN1, MAPT
10folate459.6MAPT, PSEN1, APOE
11glycerol45 24 1111.5MAPT, RPS27A, PSEN1
12streptozotocin459.5APOE, RPS27A
13thioflavine s459.4MAPT, RPS27A, APOE
14formate459.4MAPT, RPS27A, APOE
15chloroquine45 3 51 29 1113.4APOE, RPS27A, MAPT
16sodium dodecylsulfate459.4MAPT, RPS27A, APOE
17glycogen45 2410.4MAPT, RPS27A, PSEN1
18biotin45 24 1111.3RPS27A, PSEN1, APOE
19tamoxifen45 51 29 1112.3APOE, RPS27A, GRN
20n acetylcysteine459.3MAPT, RPS27A, APOE
21acetylcholine45 51 29 24 1113.2MAPT, RPS27A, APOE
22proline459.2MAPT, RPS27A, PSEN1
23actinomycin d459.2MAPT, RPS27A, APOE
24paraffin459.1MAPT, RPS27A, APOE
254-hydroxynonenal45 2410.1MAPT, RPS27A, PSEN1, APOE
26lactacystin459.1APOE, PSEN1, RPS27A, MAPT
27valine459.1APOE, PSEN1, RPS27A, MAPT
28nmda45 2910.1APOE, PSEN1, RPS27A, MAPT
29guanidine hydrochloride459.0RPS27A, APOE
30aspartate459.0APOE, PSEN1, RPS27A, MAPT
31threonine459.0MAPT, RPS27A, PSEN1, VCP
32cycloheximide459.0MAPT, RPS27A, PSEN1, APOE
33cyclosporin a45 29 6111.0APOE, RPS27A, MAPT
34h2o2459.0MAPT, RPS27A, PSEN1, APOE
35arginine459.0APOE, PSEN1, RPS27A, MAPT
36glutamate458.9APOE, PSEN1, RPS27A, MAPT
37estrogen458.9APOE, RPS27A, MAPT, GRN
38alanine458.9APOE, PSEN1, RPS27A, MAPT
39dopamine45 29 24 1111.9MAPT, RPS27A, APOE
40superoxide45 249.8TARDBP, PSEN1, VCP
41cysteine458.7GRN, MAPT, RPS27A, PSEN1, APOE
42glutamine458.6MAPT, RPS27A, PSEN1, VCP, APOE
43serine458.5MAPT, RPS27A, PSEN1, VCP, APOE

GO Terms for genes affiliated with Semantic Dementia

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Sources:
16Gene 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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Sources for Semantic Dementia

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet