Frontotemporal Dementia malady

NIH Rare Diseases: Frontotemporal dementia describes a group of conditions associated with shrinking of the frontal and temporal anterior lobes of the brain. Symptoms include either variable changes in behavior (e.g., impulsive, bored, listless, lack of social contact, lack of empathy, distractibility, blunted emotions, compulsive behavior, decreased energy and motivation) or problems with language (e.g., difficulty making or understanding speech). Spatial skills and memory remain intact. There is a strong genetic component to the disease; it often runs in families. There is no cure for frontotemporal dementia at this time, as a result treatment remains supportive.Although the name and classification of FTD has been a topic of discussion for over a century, the current classification of the syndrome groups together Pick’s disease, primary progressive aphasia, and semantic dementia as FTD. Some doctors propose adding corticobasal degeneration and progressive supranuclear palsy to FTD and calling the group Pick Complex. You can click on the links to view the GARD pages on these conditions.³⁰

MalaCards: Frontotemporal Dementia, also known as pallidopontonigral degeneration, is related to vascular dementia and frontotemporal lobar degeneration with ubiquitin-positive inclusions. An important gene associated with Frontotemporal Dementia is MAPT (microtubule-associated protein tau), and among its related pathways are Protein Stability and Cytoskeleton remodeling Neurofilaments. The drugs citalopram hydrobromide and citalopram and the compounds carbachol and thioflavine s have been mentioned in the context of this disorder. Affiliated tissues include brain, t cells and b cells, and related mouse phenotypes are endocrine/exocrine gland and other.

NINDS: 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.³¹

Genetics Home Reference: GRN-related frontotemporal dementia is a progressive brain disorder that can affect behavior, language, and movement. The symptoms of this disorder usually become noticeable in a person's fifties or sixties, and affected people typically survive 6 to 7 years after the appearance of symptoms. However, the features of this condition vary significantly, even among affected members of the same family.¹⁷

Wikipedia: Frontotemporal dementia (FTD) is the clinical syndrome caused by degeneration of the frontal lobe of the...⁴⁴ more...

OMIM: 600274

Aliases & Descriptions:

frontotemporal dementia 6 30 31 8 32 43 pallidopontonigral degeneration 6 7 43 multiple system tauopathy with presenile dementia 6 30 frontotemporal lobar degeneration 6 43 dementia, frontotemporal, with parkinsonism 30 pallidopontonigral degeneration (disorder) 6 frontotemporal dementia with parkinsonism 30

frontotemporal lobe dementia (fldem) 30 dementia, frontotemporal 33 wilhelmsen-lynch disease 6 tauopathies 43 dementia 43 mstd 30 ftd 30

Diseases related to frontotemporal dementia by text searches and GeneDecks gene sharing:

(show top 50)    (show all 359)

id	Related Disease	Score	Top Affiliating Genes
1	vascular dementia	36.9	BCHE, APOE, APP, MAPT, SNCA, RPS27A
2	frontotemporal lobar degeneration with ubiquitin-positive inclusions	36.6	GRN, LOC643387, RPS27A, TARDBP, SQSTM1
3	inclusion body myopathy	35.6	VCP, MPFD, APP, GNE, SNCA, RPS27A
4	alzheimer's disease	35.5	APOE, APP, MAPT, PSEN1, PSEN2, PRNP
5	amyotrophic lateral sclerosis with frontotemporal dementia	34.8	GRN, RPS27A, C9orf72, TARDBP, SOD1
6	paget's disease of bone	34.7	VCP, UBA52, GNE, SNCA, RPS27A, PDLIM3
7	lateral sclerosis	34.1	MAPT, SNCA, SOD1
8	lewy body dementia	34.0	PARK2, BCHE, APOE, APP, MAPT, SNCB
9	tauopathy	33.3	SERPINI1, PARK2, CDKN3, INA, GRN, APP
10	motor neuron disease	32.9	VCP, BDNF, CHMP2B, INA, GRN, APOE
11	primary progressive aphasia	32.8	GRN, MAPT, RPS27A
12	myopathy	32.2	CNBP, VCP, PARK2, BDNF, UBA52, BAX
13	binswanger's disease	32.1	APOE, APP, ACHE
14	was-related disorders	31.7	RELN, VCP, BDNF, CHGA, GRN, APOA1
15	progressive supranuclear palsy	31.7	PARK2, APOE, APP, MAPT, SNCA, RPS27A
16	aphasia	31.5	SEMA3A, CHMP2B, UBA52, FUS, GRN, APOE
17	cerebrovascular disease	31.3	BACE1, APOA1, APOE, APP, MAPT, ACHE
18	paralysis	30.9	SEMA3A, VCP, PARK2, BCHE, UBQLN2, FUS
19	immunodeficiency	30.7	PARK2, CDKN3, IL15, MAPT, CRYAB, ACHE
20	semantic dementia	30.7	GRN, APOE, MAPT, RPS27A, TARDBP
21	corticobasal degeneration	30.6	MAPT, SNCA, RPS27A, TARDBP
22	amyloidosis	30.6	PARK2, BACE1, APOA1, APOE, APP, MAPT
23	pick's disease	30.6	PCSK1N, BDNF, SCG2, CHGA, MTA2, GRN
24	cerebral amyloid angiopathy	30.4	BCHE, APOE, APP, MAPT, RPS27A, PSEN1
25	supranuclear palsy	30.4	PARK2, BCHE, MTA2, APOE, APP, MAPT
26	cerebral atrophy	30.3	APOE, MAPT, RPS27A, GFAP
27	down syndrome	29.9	BDNF, BACE1, BAX, CDKN3, APOE, APP
28	alcoholism	29.8	MAPT, MAOA, SNCA, NPY, GAL
29	differentiating neuroblastoma	29.7	BDNF, CHGA, APP, MAPT, SNCA, RPS27A
30	autonomic dysfunction	29.5	BDNF, APOA1, MAPT, SNCA, RPS27A, NPY
31	gliosis	29.4	PARK2, BACE1, APP, MAPT, SNCA, CRYAB
32	creutzfeldt-jakob syndrome	29.2	CNBP, APOE, APP, MAPT, SNCA, CRYAB
33	multiple system atrophy	29.0	PARK2, BDNF, APOE, MAPT, SUMO1, SNCB
34	normal pressure hydrocephalus	28.9	BDNF, APOE, MAPT, GFAP, NPY, GAL
35	familial idiopathic basal ganglia calcification	28.9	APP, MAPT, PSEN1, PSEN2
36	early-onset familial alzheimer disease	28.9	SERPINI1, APP, MAPT, SNCA, PSEN1
37	basal ganglia calcification	28.9	APP, MAPT, PSEN1, PSEN2
38	alzheimer disease type 2	28.8	APOE, PSEN1, PSEN2
39	homocysteine	28.7	BCHE, JUN, CDKN3, APOA1, APOE, APP
40	head injury	28.7	BDNF, APOE, APP, ACHE, GFAP, SOD1
41	inclusion body myositis	28.5	APP, MAPT, RPS27A
42	prion disease	28.5	APP, MAPT, PSEN1, PSEN2, PRNP
43	spasticity	28.5	MT-ND1, JUN, CDKN3, CDK1, IL15, MAPT
44	sleep disorder	27.9	CNBP, APOE, SNCA, PRNP, NPY
45	chorea	27.6	BDNF, RPS27A, ACHE, GFAP, TARDBP, NPY
46	amyotrophic lateral sclerosis	27.6	PCSK1N, VCP, PARK2, BDNF, SCG2, CHGB
47	parkinson's disease	27.6	VCP, PARK2, BDNF, BCHE, BAX, MT-ND1
48	memory impairment	27.5	BDNF, BCHE, BACE1, GRN, APOE, APP
49	huntington's disease	27.4	SERPINI1, BDNF, APP, MAPT, SNCA, RPS27A
50	hydrocephalus	27.4	BDNF, APOE, APP, MAPT, ACHE, GFAP

Clinical features from OMIM: 600274

Approved drugs:

Drug clinical trials:

Inferred drug relations via UMLS/NDF-RT:

^43 28 citalopram, citalopram hydrobromide, prochlorperazine, prochlorperazine edisylate, prochlorperazine maleate, risperidone

MalaCards organs/tissues related to frontotemporal dementia:

²²

MGI Mouse Phenotypes related to frontotemporal dementia:

²⁵ (show all 19)

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	endocrine/exocrine gland phenotype	MP:0005379	10.1	GAL, NEK1, PSEN2, PSEN1, LRRK2, FUS
2	other phenotype	MP:0005395	9.9	SORL1, PRNP, PSEN1, MAPT, APP, APOE
3	hematopoietic system phenotype	MP:0005397	9.5	NEK1, PML, SQSTM1, LRRK2, MAP3K14, GRN
4	no phenotypic analysis	MP:0003012	9.1	SNCA, LRRK2, ACHE, TARDBP, PRNP, PML
5	muscle phenotype	MP:0005369	9.1	CRYAB, PDLIM3, ACHE, GFAP, PSEN1, PRNP
6	reproductive system phenotype	MP:0005389	8.9	SNCA, ACHE, PRNP, NEK1, ADAMTS2, GAL
7	skeleton phenotype	MP:0005390	8.6	NEK1, SQSTM1, CRYAB, SNCA, ISG15, JUN
8	immune system phenotype	MP:0005387	8.6	GFAP, DNTT, PSEN1, PSEN2, PRNP, SQSTM1
9	vision/eye phenotype	MP:0005391	8.4	ACHE, GFAP, PSEN1, PSEN2, SOD1, SORT1
10	integument phenotype	MP:0010771	8.0	MAP3K14, SNCA, LRRK2, PSEN1, PSEN2, SQSTM1
11	renal/urinary system phenotype	MP:0005367	7.9	APOE, GSK3B, MTA2, BAX, CHGA, MAPT
12	nervous system phenotype	MP:0003631	7.8	RELN, ACHE, PSEN1, SQSTM1, SORL1, NPY
13	cardiovascular system phenotype	MP:0005385	7.5	WNT2, SORL1, SOD1, PSEN2, CHGA, YWHAQ
14	normal phenotype	MP:0002873	7.1	GFAP, DNTT, PSEN1, PSEN2, PRNP, PML
15	behavior/neurological phenotype	MP:0005386	6.1	TARDBP, GFAP, ACHE, LRRK2, CRYAB, SNCA
16	cellular phenotype	MP:0005384	5.3	PSEN1, DCTN1, TARDBP, DNTT, GFAP, LRRK2
17	growth/size phenotype	MP:0005378	5.1	GFAP, ACHE, CRYAB, SNCA, SNCB, TRA2B
18	homeostasis/metabolism phenotype	MP:0005376	4.9	PSEN1, TARDBP, GFAP, ACHE, LRRK2, PDLIM3
19	mortality/aging	MP:0010768	4.4	ACHE, LRRK2, PDLIM3, CRYAB, SNCA, SNCB

Articles related to frontotemporal dementia:

(show top 50)    (show all 244)

id	Title	Authors	Year	Affiliating Genes
1	Cerebrospinal fluid tau, p-tau 181 and amyloid-I^38/40 /42 in frontotemporal dementias and primary progressive aphasias. (21135556)	Bibl M.... Wiltfang J.	2011	APP, MAPT
2	The APOE gene locus in frontotemporal dementia and pr imary progressive aphasia. (21555637)	Seripa D.... Masullo C.	2011	APOE
3	The frontotemporal dementia mutation R406W blocks tau 's interaction with the membrane in an annexin A2-dependent manner. (21339331)	Gauthier-Kemper A.... Brandt R.	2011	MAPT
4	Frontotemporal dementia and parkinsonism linked to ch romosome 17--the first Polish family. (20561037)	NaroA1aA8ska E.... SA8awek J.	2011	MAPT
5	Two Australian families with inclusion-body myopathy, Paget's disease of bone and frontotemporal dementia: novel clinical and geneti c findings. (20335036)	Kumar K.R.... Mastaglia F.L.	2010	VCP
6	Heterogeneous nuclear ribonucleoprotein E3 modestly a ctivates splicing of tau exon 10 via its proximal downstream intron, a hotspot for frontotemporal dementia mutations. (19914360)	Wang Y.... Andreadis A.	2010	MAPT, PCBP3
7	Plasma progranulin levels predict progranulin mutation status in frontotemporal dementia patients and asymptomatic family members. (19158106)	Finch N.... Rademakers R.	2009	GRN, RPS27A
8	Inclusion body myopathy and frontotemporal dementia caused by a novel VCP mutation. (17889967)	Bersano A.... Corti S.	2009	VCP
9	Clinical outcome in 19 French and Spanish patients with valosin-containing protein myopathy associated with Paget's disease of bone and frontotemporal dementia. (19364651)	Stojkovic T.... Eymard B.	2009	VCP
10	Association of GSK3B with Alzheimer disease and frontotemporal dementia. (18852354)	Schaffer B.A.... Geschwind D.H.	2008	GSK3B, MAPT
11	The functional MAOA-uVNTR promoter polymorphism in patients with frontotemporal dementia. (18474080)	Reif A.... Galimberti D.	2008	MAOA
12	Frontotemporal dementia and Parkinsonism linked to chromosome 17 with the N279K tau mutation. (17319286)	Slowinski J.... Wszolek Z.K.	2007	MAPT
13	Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy. (17457594)	Guinto J.B.... Forman M.S.	2007	VCP, RPS27A
14	Preliminary findings: behavioral worsening on donepezil in patients with frontotemporal dementia. (17194818)	Mendez M.F.... Licht E.	2007	ACHE
15	Structural and microtubule binding properties of tau mutants of frontotemporal dementias. (17297915)	Fischer D.... Zweckstetter M.	2007	MAPT
16	Clinicopathologic features of frontotemporal dementia with progranulin sequence variation. (17202431)	Spina S.... Grafman J.	2007	GRN, RPS27A
17	No association of chromatin-modifying protein 2B with sporadic frontotemporal dementia. (16979267)	Schumacher A.... Riemenschneider M.	2007	CHMP2B
18	Heterogeneity within a large kindred with frontotemporal dementia: a novel progranulin mutation. (17620546)	Bruni A.C.... Rogaeva E.	2007	GRN
19	Biochemical and pathological characterization of frontotemporal dementia due to a Leu266Val mutation in microtubule-associated protein tau in an African American individual. (17072625)	Van Deerlin V.M.... Chatterjee A.	2007	MAPT
20	Dysregulation of tau phosphorylation is a hypothesized point of convergence in the pathogenesis of alzheimer's disease, frontotemporal dementia and schizophrenia with therapeutic implications. (16793187)	Deutsch S.I.... Lakshman R.M.	2006	MAPT, RELN
21	Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. (16862116)	Baker M.... Hutton M.	2006	MAPT, WNT2, GRN
22	Comparison of extent of tau pathology in patients with frontotemporal dementia with Parkinsonism linked to chromosome 17 (FTDP-17), frontotemporal lobar degeneration with Pick bodies and early onset Alzheimer's disease. (16866983)	Shiarli A.M.... Mann D.M.	2006	MAPT
23	Frontotemporal dementia--Part II. Differential diagnosis, genetics, molecular pathomechanism and pathology. (16173270)	Galariotis V.... KA!lmA!n J.	2005	MAPT
24	A new family with frontotemporal dementia with intronic 10+3 splice site mutation in the tau gene: neuropathology and molecular effects. (16008820)	Neumann M.... Kretzschmar H.A.	2005	MAPT
25	APOE and modulation of Alzheimer's and frontotemporal dementia. (15036621)	Boccardi M.... Frisoni G.B.	2004	APOE
26	The tau gene locus and frontotemporal dementia. (15178931)	Pickering-Brown S.	2004	MAPT
27	Chromosomal translocation t(18;21)(q23;q22.1) indicat es novel susceptibility loci for frontotemporal dementia with ALS. (14705124)	Prudlo J.... Meyer T.	2004	SOD1
28	Genetic tau-variants in patients with frontotemporal dementia (15570502)	Ibach B.... Hajak G.	2004	MAPT
29	The L266V tau mutation is associated with frontotemporal dementia and Pick-like 3R and 4R tauopathy. (12883828)	Hogg M.... Bigio E.H.	2003	MAPT
30	A novel L266V mutation of the tau gene causes frontotemporal dementia with a unique tau pathology. (12509859)	Kobayashi T.... Mori H.	2003	MAPT
31	Increased frequency of a new polymorphism in the cell division cycle 2 (cdc2) gene in patients with Alzheimer's disease and frontotemporal dementia. (12648761)	Johansson A.... Blennow K.	2003	CDK1
32	Selective reduction of soluble tau proteins in sporadic and familial frontotemporal dementias: an international follow-up study. (12677447)	Zhukareva V.... Lee V.M.	2003	MAPT
33	Polymorphisms in the tau gene in sporadic frontotemporal dementia and other neurodegenerative disorders. (12220379)	Panegyres P.K.... Zafiris-Toufexis K.	2002	MAPT
34	Microtubule associated protein (tau) gene variability in patients with frontotemporal dementia. (12121033)	Kowalska A.... Tabira T.	2002	MAPT
35	Late-onset frontotemporal dementia with a novel exon 1 (Arg5His) tau gene mutation. (11921059)	Hayashi S.... Takahashi H.	2002	MAPT
36	Apolipoprotein E gene in frontotemporal dementia: an association study and meta-analysis. (12107813)	Verpillat P.... Clerget-Darpoux F.	2002	APOE
37	Familial frontotemporal dementia and parkinsonism with a novel N296H mutation in exon 10 of the tau gene and a widespread tau accumulation in the glial cells. (11585254)	Iseki E.... Kosaka K.	2001	MAPT
38	Familial frontotemporal dementia with ubiquitin-positive inclusions is linked to chromosome 17q21-22. (11571213)	Rosso S.M.... van Swieten J.C.	2001	MAPT, SNCA
39	Tau mutations in frontotemporal dementia FTDP-17 and their relevance for Alzheimer's disease. (10899436)	Goedert M.... Spillantini M.G.	2000	MAPT
40	Tau gene mutations in frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). Their relevance for understanding the neurogenerative process. (11193179)	Goedert M.... Spillantini M.G.	2000	MAPT
41	Familial frontotemporal dementia with ubiquitin inclu sion bodies and without motor neuron disease. (10985702)	KAPvari E.... Bouras C.	2000	RPS27A
42	High prevalence of mutations in the microtubule-associated protein tau in a population study of frontotemporal dementia in the Netherlands. (9973279)	Rizzu P.... Heutink P.	1999	MAPT
43	Frontotemporal dementia and corticobasal degeneration in a family with a P301S mutation in tau. (10374757)	Bugiani O.... Ghetti B.	1999	MAPT
44	Effects of frontotemporal dementia FTDP-17 mutations on heparin-induced assembly of tau filaments. (10359094)	Goedert M.... Crowther R.A.	1999	MAPT
45	Molecular genetic characterisation of frontotemporal dementia on chromosome 3. (10436350)	Ashworth A.... Collinge J.	1999	CHMP2B
46	From genotype to phenotype: a clinical pathological, and biochemical investigation of frontotemporal dementia and parkinsonism (FTDP-17) caused by the P301L tau mutation. (10360762)	Nasreddine Z.S.... Geschwind D.H.	1999	MAPT
47	Structure of tau exon 10 splicing regulatory element RNA and destabilization by mutations of frontotemporal dementia and parkinsonism linked to chromosome 17. (10393977)	Varani L.... Varani G.	1999	MAPT
48	A clinical pathological comparison of three families with frontotemporal dementia and identical mutations in the tau gene (P301L) (10219785)	Bird T.D.... Schellenberg G.D.	1999	MAPT
49	Tau, ubiquitin, and alpha B-crystallin immunohistochemistry define the principal causes of degenerative frontotemporal dementia. (7575218)	Cooper P.N.... Mann D.M.	1995	CRYAB, RPS27A
50	Inclusion Body Myopathy with Pa get Disease of Bone and/or Frontotemporal Dementia (20301649)	Kimonis V.... Watts G.	1993	VCP

Genes related to frontotemporal dementia according to GeneCards:

(show top 50)    (show all 92)

id	Symbol	Description	Score	GeneCards Section Context
1	MAPT	microtubule-associated protein tau	11.1	Summaries, Publications
2	C9orf72	chromosome 9 open reading frame 72	11.1	Summaries, Publications
3	GRN	granulin	11.0	Orthologs, Publications
4	VCP	valosin containing protein	11.0	Publications
5	CHMP2B	charged multivesicular body protein 2B	11.0	Publications
6	TARDBP	TAR DNA binding protein	11.0	Publications
7	RPS27A	ribosomal protein S27a	11.0	Publications
8	SNCA	synuclein, alpha (non A4 component of amyloid precursor)	11.0	Publications
9	FUS	fused in sarcoma	11.0
10	SOD1	superoxide dismutase 1, soluble	11.0	Publications
11	MPFD	myopathy with fiber type disproportion	11.0
12	STH	saitohin	11.0	Publications
13	INA	internexin neuronal intermediate filament protein, alpha	11.0
14	ISG15	ISG15 ubiquitin-like modifier	11.0
15	PSEN1	presenilin 1	11.0	Publications
16	GAP43	growth associated protein 43	11.0	Publications
17	CRYAB	crystallin, alpha B	11.0	Publications
18	PIN1	peptidylprolyl cis/trans isomerase, NIMA-interacting 1	11.0	Publications
19	SERPINI1	serpin peptidase inhibitor, clade I (neuroserpin), member 1	11.0
20	PSEN2	presenilin 2 (Alzheimer disease 4)	11.0	Publications
21	GNE	glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase	11.0	Publications
22	SQSTM1	sequestosome 1	11.0	Publications
23	ACHE	acetylcholinesterase	11.0	Publications
24	APOE	apolipoprotein E	11.0	Publications
25	BACE1	beta-site APP-cleaving enzyme 1	11.0
26	RELN	reelin	11.0	Publications
27	APP	amyloid beta (A4) precursor protein	11.0	Publications
28	CDK1	cyclin-dependent kinase 1	11.0	Publications
29	LRRK2	leucine-rich repeat kinase 2	11.0
30	PARK2	parkinson protein 2, E3 ubiquitin protein ligase (parkin)	11.0
31	MIR659	microRNA 659	11.0	Publications
32	MIR29B2	microRNA 29b-2	11.0	Publications
33	LOC643387	TAR DNA binding protein pseudogene	11.0	Publications
34	PCSK1N	proprotein convertase subtilisin/kexin type 1 inhibitor	11.0	Proteins, Publications
35	MIR29B1	microRNA 29b-1	11.0	Publications
36	UBQLN2	ubiquilin 2	11.0
37	RUNDC3A	RUN domain containing 3A	11.0
38	CELF3	CUGBP, Elav-like family member 3	11.0	Publications
39	FMNL1	formin-like 1	11.0	Publications
40	SRSF9	serine/arginine-rich splicing factor 9	11.0	Publications
41	SNCB	synuclein, beta	11.0	Publications
42	KLK10	kallikrein-related peptidase 10	11.0	Publications
43	SCG2	secretogranin II	11.0	Publications
44	NEFH	neurofilament, heavy polypeptide	11.0	Publications
45	NEFL	neurofilament, light polypeptide	11.0	Publications
46	CHGB	chromogranin B (secretogranin 1)	11.0	Publications
47	KLK7	kallikrein-related peptidase 7	11.0	Publications
48	TRA2B	transformer 2 beta homolog (Drosophila)	11.0	Publications
49	PDLIM3	PDZ and LIM domain 3	11.0	Publications
50	WNT2	wingless-type MMTV integration site family member 2	11.0	Publications

Pathways related to frontotemporal dementia according to GeneDecks:

(show all 13)

id	Pathway	Score	Top Affiliating Genes
1	Protein Stability3	10.1	SQSTM1, SUMO1, ISG15, ATG7, PARK2, VCP
2	Cytoskeleton remodeling Neurofilaments10	10.0	NEFL, NEFH, DCTN1, GFAP, INA
3	Cytoskeleton remodeling_Neurofilaments41	10.0	NEFL, NEFH, DCTN1, GFAP, INA
4	Alzheimers Disease Pathway36	10.0	PSEN2, PSEN1, MAPT, APP, APOE, GSK3B
5	Processing of Capped Intron-Containing Pre-mRNA38	9.9	SRSF9, SRSF7, SRSF6, SRSF4, SRSF11, PTBP1
6	Alzheimers disease20	9.8	PSEN2, PSEN1, SNCA, MAPT, APP, APOE
7	Spliceosome20	9.6	SRSF9, SRSF7, SRSF6, SRSF4, TRA2B, U2AF2
8	Neuroscience3	9.4	PARK2, GAP43, NEFL, NEFH, SOD1, PSEN2
9	Proteolysis_Putative SUMO-1 pathway41	9.4	UBA52, JUN, SUMO1, PML
10	Reelin Pathway (Cajal-Retzius cells)36	9.3	WNT2, MAPT, APP, APOE, GSK3B, JUN
11	Proteolysis Putative SUMO-1 pathway10	9.3	PML, SUMO1, JUN, UBA52
12	DHA Signaling36	8.4	MAPT, APP, GSK3B, BAX, YWHAQ, BDNF
13	Neurotrophin signaling pathway20	8.3	SORT1, PSEN2, PSEN1, GSK3B, JUN, BAX

Compounds related to frontotemporal dementia according to GeneDecks:

(show top 50)    (show all 122)

id	Compound	Score	Top Affiliating Genes
1	carbachol32 9 9	12.8	SNCA
2	thioflavine s32	10.8	MAPT, SNCA, APP, RPS27A, APOE
3	tacrine32 9 9	12.4	APP, MAPT, BCHE, ACHE, APOE
4	formate32	10.3	SOD1, GFAP, APP, RPS27A, APOE, PRNP
5	secretoneurin32	10.1	NPY, CHGA, GAL, SCG2, CHGB
6	lysine32	9.9	SUMO1, PSEN2, PRNP, PML, BACE1, PSEN1
7	selegiline32 9 9	11.8	MAOA, SOD1, BDNF, ACHE, SNCA, BCHE
8	cysteine32	9.8	GRN, RPS27A, GAP43, MAPT, PML, PSEN1
9	6-hydroxydopamine32	9.7	NPY, APP, BDNF, PARK2, MAOA, SNCA
10	4-hydroxynonenal32 18	10.7	APP, BACE1, JUN, APOE, RPS27A, BAX
11	dopamine32 9 18 9	12.7	ACHE, GFAP, GAP43, SCG2, SNCA, MAOA
12	threonine32	9.6	NEK1, MT-ND1, MAPT, PSEN1, GAL, GAP43
13	aspartate32	9.6	APOE, APOA1, BACE1, BDNF, PIN1, DNTT
14	lactacystin32	9.6	JUN, APOE, APP, MAPT, SNCA, RPS27A
15	superoxide32 18	10.5	NEFH, PARK2, VCP, SOD1, NEFL, BACE1
16	glutamine32	9.4	PML, VCP, CRYAB, APOE, APP, MAPT
17	catecholamine32	9.4	GAP43, NPY, GAL, SNCA, MAOA, CHGA
18	glutamate32	9.3	MAOA, GAP43, GAL, BCHE, NEFL, SORT1
19	levodopa32 9 9	11.3	MAOA, BDNF, GAL, SOD1, CHGA, LRRK2
20	norepinephrine32 9 18 9	12.2	BCHE, GAL, BDNF, CHGB, APP, SCG2
21	valine32	9.2	APP, BDNF, MT-ND1, CDK1, APOE, MAPT
22	paraffin32	9.1	CDK1, APOE, MAPT, BAX, SNCA, PSEN2
23	choline32 9 18 9	12.0	APP, MAPT, MAOA, SNCA, ACHE, GFAP
24	streptozotocin32	8.9	APOE, BDNF, CHGA, CDKN3, NPY, GFAP
25	nmda32 42	9.9	CDKN3, GAP43, BCHE, BDNF, PARK2, APOE
26	mptp32	8.9	RPS27A, PARK2, BDNF, BAX, MAOA, SNCA
27	kainate32	8.8	NPY, JUN, BDNF, CDKN3, APOE, MAPT
28	formaldehyde32 18	9.8	APOA1, CDKN3, PRNP, APP, MAPT, RPS27A
29	gaba32 42	9.7	RELN, GAL, NPY, GFAP, RPS27A, GAP43
30	cholesterol32 9 18 9	11.7	PSEN1, GAP43, PSEN2, RELN, DNTT, GFAP
31	methionine32	8.7	GAL, CHGA, MAPT, SNCA, MT-ND1, PRNP
32	glycogen32 18	9.6	RELN, WNT2, MAPT, CHGA, CDKN3, CDK1
33	arginine32	8.6	NPY, SOD1, PRNP, PSEN1, GFAP, SNCA
34	acetylcholine32 9 18 9	11.4	CHGA, BDNF, CDKN3, BCHE, BACE1, RPS27A
35	estrogen32	8.4	RPS27A, SUMO1, GFAP, KLK10, PSEN2, NPY
36	creatinine32	8.4	NEFL, NPY, PDLIM3, RPS27A, APP, MAPT
37	camptothecin32 42 9 9	11.3	RPS27A, SUMO1, ISG15, CDK1, CDKN3, BAX
38	alanine32	8.3	IL15, GFAP, NPY, KLK10, MASP2, MAP3K14
39	adenylate32	8.3	GAP43, GAL, DNTT, GFAP, RPS27A, SCG2
40	zinc32 18	9.1	GFAP, CDK1, PML, SOD1, SQSTM1, PRNP
41	oxygen32 18	9.0	PSEN1, MAOA, IL15, CDK1, JUN, BAX
42	cycloheximide32	7.9	APOE, GAP43, GAL, PSEN1, GFAP, RPS27A
43	lipid32	7.6	PARK2, LRRK2, GFAP, GSK3B, PSEN1, GAP43
44	testosterone32 9 18 9	10.5	PARK2, CDKN3, CHGA, BDNF, APP, CDK1
45	vegf32	7.2	SEMA3A, RPS27A, CHGA, GFAP, WNT2, BAX
46	h2o232	7.1	GAP43, MAP3K14, MAPT, APP, APOE, APOA1
47	atp32	6.7	ISG15, CDK1, CDKN3, CHGA, SCG2, BDNF
48	retinoic acid32 42 18	8.4	NPY, GFAP, SUMO1, MAPT, BDNF, CHGA
49	tyrosine32	6.1	CDK1, JUN, BACE1, CHGA, CDKN3, CHGB
50	serine32	4.8	GAP43, SRSF7, MAP4K4, MASP2, MAP3K14, MAPT

Cellular components related to frontotemporal dementia according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	neurofilament	GO:005883	10.2	INA, NEFH, NEFL
2	axon	GO:030424	9.9	GAP43, NEFL, NEFH, SNCA, MAPT, APP
3	secretory granule	GO:030141	9.7	GAL, APOA1, CHGA, CHGB, SCG2, PCSK1N
4	Golgi apparatus	GO:005794	9.3	PSEN1, PSEN2, PRNP, SORT1, GAL, ACHE
5	nucleoplasm	GO:005654	8.6	SRSF4, SRSF6, SRSF7, SRSF9, RPS27A, SQSTM1
6	nucleolus	GO:005730	7.7	SRSF7, SNCB, DNTT, TARDBP, PRNP, SOD1
7	extracellular region	GO:005576	7.5	SNCA, KLK10, KLK7, ACHE, SOD1, NPY
8	cytosol	GO:005829	7.5	MAP3K14, GNE, SNCA, CRYAB, RPS27A, GFAP
9	cytoplasm	GO:005737	6.3	WNT2, IFT74, GNE, SUMO1, SNCB, SNCA
10	nucleus	GO:005634	5.7	SNCA, SNCB, SRSF7, SRSF6, SRSF4, SRSF11

Biological processes related to frontotemporal dementia according to GeneDecks:

(show all 24)

id	Name	GO ID	Score	Top Affiliating Genes
1	neurofilament cytoskeleton organization	GO:060052	10.6	NEFH, SOD1, INA
2	Notch receptor processing	GO:007220	10.6	PSEN2, PSEN1, RPS27A, UBA52
3	positive regulation of nuclear mRNA splicing, via spliceosome	GO:048026	10.4	CELF3, CELF4, TRA2B
4	negative regulation of type I interferon production	GO:032480	10.4	PIN1, UBA52, ISG15, RPS27A
5	endoplasmic reticulum calcium ion homeostasis	GO:032469	10.4	PML, PSEN2, PSEN1
6	positive regulation of proteasomal ubiquitin-dependent protein catabolic process	GO:032436	10.4	VCP, SUMO1, LRRK2, PSEN1
7	cell death	GO:008219	10.3	NEFH, DCTN1, TARDBP, C9orf72, APOE, FUS
8	regulation of alternative nuclear mRNA splicing, via spliceosome	GO:000381	10.3	PTBP1, TRA2B, CELF4, CELF3
9	activation of MAPK activity	GO:000187	10.3	UBA52, CDK1, RPS27A, LRRK2, SOD1
10	mRNA splice site selection	GO:006376	10.2	SRSF9, SRSF6, CELF4
11	negative regulation of nuclear mRNA splicing, via spliceosome	GO:048025	10.2	U2AF2, PTBP1, SRSF4, SRSF6, SRSF7, SRSF9
12	mRNA 3-end processing	GO:031124	10.2	SRSF9, SRSF7, SRSF6, SRSF4, SRSF11, U2AF2
13	termination of RNA polymerase II transcription	GO:006369	10.1	SRSF9, SRSF7, SRSF6, SRSF4, SRSF11, U2AF2
14	mRNA export from nucleus	GO:006406	10.1	SRSF9, SRSF7, SRSF6, SRSF4, SRSF11, U2AF2
15	induction of apoptosis by extracellular signals	GO:008624	9.9	SORT1, SQSTM1, PSEN2, PSEN1, RPS27A, BAX
16	stress-activated MAPK cascade	GO:051403	9.9	RPS27A, CRYAB, CDK1, JUN, UBA52
17	RNA splicing	GO:008380	9.9	TARDBP, SRSF9, SRSF7, SRSF6, SRSF4, SRSF11
18	nuclear mRNA splicing, via spliceosome	GO:000398	9.9	SRSF9, SRSF7, SRSF6, SRSF4, SRSF11, PTBP1
19	nerve growth factor receptor signaling pathway	GO:048011	9.7	SORT1, SQSTM1, PSEN2, PSEN1, RPS27A, GSK3B
20	mRNA processing	GO:006397	9.7	TARDBP, SRSF9, SRSF7, SRSF4, SRSF11, PTBP1
21	positive regulation of protein complex assembly	GO:031334	9.4	SUMO1, GSK3B, VCP
22	negative regulation of neuron apoptotic process	GO:043524	9.3	SOD1, PSEN1, SNCA, SNCB, JUN, BAX
23	anti-apoptosis	GO:006916	9.2	RPS27A, PSEN1, PRNP, SQSTM1, SOD1, CRYAB
24	response to drug	GO:042493	8.6	GAL, SOD1, SNCA, GSK3B, CDK1, JUN

Molecular functions related to frontotemporal dementia according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	beta-amyloid binding	GO:001540	9.8	ACHE, APOE, APOA1, BCHE
2	tau protein binding	GO:048156	9.5	GSK3B, APOE, SNCA
3	RNA binding	GO:003723	9.5	SRSF4, SRSF6, SRSF7, SRSF9, SRSF11, PTBP1
4	identical protein binding	GO:042802	9.2	NEFL, SQSTM1, PRNP, SNCA, APP, APOE
5	nucleotide binding	GO:000166	9.2	SRSF4, SRSF6, SRSF7, SRSF9, TARDBP, SRSF11
6	protein binding	GO:005515	4.2	GAP43, CRYAB, SNCA, SRSF7, SRSF11, SUMO1