MCID: GRN014
MIFTS: 37

Grn-Related Frontotemporal Dementia

Categories: Mental diseases, Genetic diseases, Rare diseases, Neuronal diseases

Aliases & Classifications for Grn-Related Frontotemporal Dementia

MalaCards integrated aliases for Grn-Related Frontotemporal Dementia:

Name: Grn-Related Frontotemporal Dementia 24 25 73
Hereditary Dysphasic Disinhibition Dementia 24 25
Frontotemporal Lobar Degeneration 25 73
Ftdu-17 24 25
Frontotemporal Lobar Degeneration with Tdp43 Inclusions, Grn-Related 73
Ftld with Tdp-43 Pathology 25
Frontotemporal Dementia 73
Ftdp-17 Grn 25
Ftd-Pgrn 25
Ftld-Tdp 25
Ftd-Grn 25
Hddd1 25
Hddd2 25
Ftld 25

Characteristics:

GeneReviews:

24
Penetrance Penetrance is about 90% by age 75 years, but apparent incomplete penetrance has also been observed in a few cases [cruts et al 2006a, gass et al 2006]. more reports will be needed before the penetrance can be more accurately established...

Classifications:



External Ids:

Summaries for Grn-Related Frontotemporal Dementia

Genetics Home Reference : 25 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.

MalaCards based summary : Grn-Related Frontotemporal Dementia, also known as hereditary dysphasic disinhibition dementia, is related to frontotemporal dementia and frontotemporal dementia and/or amyotrophic lateral sclerosis 1, and has symptoms including personality changes, agitation and memory loss. The drugs Dopamine and Memantine have been mentioned in the context of this disorder. Affiliated tissues include brain, testes and prefrontal cortex.

GeneReviews: NBK1371

Related Diseases for Grn-Related Frontotemporal Dementia

Diseases in the Frontotemporal Dementia family:

Grn-Related Frontotemporal Dementia Chmp2b-Related Frontotemporal Dementia

Diseases related to Grn-Related Frontotemporal Dementia via text searches within MalaCards or GeneCards Suite gene sharing:

# Related Disease Score Top Affiliating Genes
1 frontotemporal dementia 11.7
2 frontotemporal dementia and/or amyotrophic lateral sclerosis 1 11.4
3 frontotemporal lobar degeneration with tdp43 inclusions, grn-related 11.4
4 dementia 10.2
5 frontotemporal dementia with parkinsonism-17 10.2

Comorbidity relations with Grn-Related Frontotemporal Dementia via Phenotypic Disease Network (PDN):


Acute Cystitis Alzheimer Disease

Graphical network of the top 20 diseases related to Grn-Related Frontotemporal Dementia:



Diseases related to Grn-Related Frontotemporal Dementia

Symptoms & Phenotypes for Grn-Related Frontotemporal Dementia

UMLS symptoms related to Grn-Related Frontotemporal Dementia:


personality changes, agitation, memory loss, restlessness

Drugs & Therapeutics for Grn-Related Frontotemporal Dementia

Drugs for Grn-Related Frontotemporal Dementia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 127)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dopamine Approved Phase 4,Phase 3,Phase 2,Phase 1 51-61-6, 62-31-7 681
2
Memantine Approved, Investigational Phase 4,Phase 3,Phase 2 19982-08-2 4054
3
Buprenorphine Approved, Illicit, Investigational, Vet_approved Phase 4 52485-79-7 40400 644073
4
Acetaminophen Approved Phase 4 103-90-2 1983
5
Citalopram Approved Phase 4 59729-33-8 2771
6
Iodine Approved, Investigational Phase 4 7553-56-2 807
7
Corticosterone Experimental Phase 4,Phase 1,Early Phase 1 50-22-6 5753
8 Fluorodeoxyglucose F18 Phase 4,Phase 2,Phase 1,Not Applicable
9 Neurotransmitter Agents Phase 4,Phase 3,Phase 2
10 Dopamine Agents Phase 4,Phase 3,Phase 2,Phase 1
11 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 1
12 Antiparkinson Agents Phase 4,Phase 3,Phase 2
13 Radiopharmaceuticals Phase 4,Phase 2,Not Applicable
14 Analgesics Phase 4,Phase 1
15 Central Nervous System Depressants Phase 4,Phase 3,Phase 2,Phase 1
16 Serotonin Agents Phase 4,Phase 3
17 Narcotic Antagonists Phase 4
18 Serotonin Uptake Inhibitors Phase 4
19 Narcotics Phase 4
20 Analgesics, Non-Narcotic Phase 4,Phase 1
21 Analgesics, Opioid Phase 4
22 Neurotransmitter Uptake Inhibitors Phase 4
23 Excitatory Amino Acid Antagonists Phase 4,Phase 3,Phase 2
24 Excitatory Amino Acids Phase 4,Phase 3,Phase 2
25 Antidepressive Agents Phase 4,Phase 2,Phase 1
26 Antidepressive Agents, Second-Generation Phase 4
27 Anti-Infective Agents Phase 4,Phase 3,Phase 1,Phase 2
28 Antipyretics Phase 4
29 Psychotropic Drugs Phase 4,Phase 3,Phase 2,Phase 1
30 Antiviral Agents Phase 4,Phase 3,Phase 1,Phase 2
31 Pharmaceutical Solutions Phase 4,Phase 2,Phase 3,Phase 1
32 Anti-Inflammatory Agents Phase 4,Phase 1
33 cadexomer iodine Phase 4
34
Serotonin Investigational, Nutraceutical Phase 4,Phase 3 50-67-9 5202
35 Calamus Nutraceutical Phase 4
36
Methylene blue Approved, Investigational Phase 3 61-73-4
37
Miglustat Approved Phase 3,Phase 2,Phase 1 72599-27-0 51634
38
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
39
Busulfan Approved, Investigational Phase 2, Phase 3 55-98-1 2478
40
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 5755
41
Benzocaine Approved, Investigational Phase 3 1994-09-7, 94-09-7 2337
42
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 6741
43
Pimavanserin Approved, Investigational Phase 3 706779-91-1 16058810
44
Deferiprone Approved Phase 2, Phase 3 30652-11-0 2972
45
Iron Approved Phase 2, Phase 3 7439-89-6 23925
46 tannic acid Approved, Nutraceutical Phase 3
47
1-Deoxynojirimycin Experimental Phase 3,Phase 2 19130-96-2 1374
48 Prednisolone acetate Phase 2, Phase 3
49 Alkylating Agents Phase 2, Phase 3
50 Methylprednisolone acetate Phase 2, Phase 3

Interventional clinical trials:

(show top 50) (show all 171)
# Name Status NCT ID Phase Drugs
1 Serotonergic Function and Behavioural and Psychological Symptoms of Frontotemporal Dementia Completed NCT00376051 Phase 4 Citalopram
2 Memantine (10mg BID) for the Frontal and Temporal Subtypes of Frontotemporal Dementia Completed NCT00545974 Phase 4 memantine;Placebo pill
3 A 52 Week Open Label Trial of Memantine for Frontotemporal Lobar Degeneration Completed NCT00187525 Phase 4 Memantine
4 Efficacy of Pain Treatment on Depression in Patients With Dementia Completed NCT02267057 Phase 4 Paracetamol;Buprenorphine;Paracetamol placebo;Buprenorphine placebo
5 Longitudinal Multi-Modality Imaging in Progressive Apraxia of Speech Recruiting NCT01818661 Phase 4 AV-1451
6 Imaging of Brain Amyloid Plaques in the Aging Population Enrolling by invitation NCT00950430 Phase 4 Pittsburgh Compound B (C-11 PiB);F-18 FDG;Tau (18-F-AV-1451)
7 DaTSCAN Imaging in Aging and Neurodegenerative Disease Enrolling by invitation NCT01453127 Phase 4 I-123 Ioflupane solution injection prior to SPECT scan (DaTscan)
8 Amantadine for the Treatment of Behavioral Disturbance in Frontotemporal Dementia (FTD) Withdrawn NCT00127114 Phase 4 Amantadine;Placebo
9 Safety and Efficacy Study Evaluating TRx0237 in Subjects With Behavioral Variant Frontotemporal Dementia (bvFTD) Completed NCT01626378 Phase 3 TRx0237;Placebo
10 Open Label Pilot Study of the Effects of Memantine on FDG-PET in Frontotemporal Dementia Completed NCT00594737 Phase 3 memantine hydrochloride
11 Application of Miglustat in Patients With Niemann-Pick Type C Completed NCT01760564 Phase 3 Miglustat
12 Stem Cell Transplant for Inborn Errors of Metabolism Completed NCT00176904 Phase 2, Phase 3 Busulfan, Cyclophosphamide, Antithymocyte Globulin
13 Efficacy, Safety, Pharmacodynamic, and Pharmacokinetics Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency Recruiting NCT02004691 Phase 2, Phase 3 placebo (saline);GZ402665
14 Relapse Prevention Study of Pimavanserin in Dementia-related Psychosis Recruiting NCT03325556 Phase 3 Placebo;Pimavanserin 34 mg;Pimavanserin 20 mg
15 Arimoclomol Prospective Study in Patients Diagnosed With NiemannPick Disease Type C Active, not recruiting NCT02612129 Phase 2, Phase 3 arimoclomol;Placebo
16 Study of VTS-270 (2-hydroxypropyl-β-cyclodextrin) to Treat Niemann-Pick Type C1 (NPC1) Disease Active, not recruiting NCT02534844 Phase 2, Phase 3 VTS-270;Sham Procedure Control
17 SurVival of Lysosomal Acid Lipase Deficiency (LAL-D) Infants Treated With SebelipAse aLfa Active, not recruiting NCT01371825 Phase 2, Phase 3 Sebelipase alfa (SBC-102)
18 Conservative Iron Chelation as a Disease-modifying Strategy in Amyotrophic Lateral Sclerosis Not yet recruiting NCT03293069 Phase 2, Phase 3 Deferiprone;Placebo Oral Tablet
19 Open-Label Study of TRx0237 in Subjects With Alzheimer's Disease or Behavioral Variant Frontotemporal Dementia (bvFTD) Terminated NCT02245568 Phase 3 TRx0237
20 ALD-101 Adjuvant Therapy of Unrelated Umbilical Cord Blood Transfusion (UCBT) in Patients With Inherited Metabolic Diseases Terminated NCT00654433 Phase 3
21 Children With Lysosomal Acid Lipase Deficiency Who Previously Received Treatment With SBC-102 Terminated NCT01473875 Phase 2, Phase 3 SBC-102
22 Effects of Tolcapone on Frontotemporal Dementia Unknown status NCT00604591 Phase 2 Tolcapone;Placebo
23 Study to Assess the Safety, Tolerability, and Pharmacodynamic (PD) Effects of FRM-0334 in Subjects With Prodromal to Moderate Frontotemporal Dementia With Granulin Mutation Unknown status NCT02149160 Phase 2 FRM-0334;Placebo
24 Phase 1/2 Study of Vorinostat Therapy in Niemann-Pick Disease, Type C1 Completed NCT02124083 Phase 1, Phase 2 Vorinostat
25 Biomarker Validation for Niemann-Pick Disease, Type C: Safety and Efficacy of N-Acetyl Cysteine Completed NCT00975689 Phase 1, Phase 2 N-Acetyl Cysteine
26 A Pilot Study to Explore the Safety and Tolerability of Galantamine HBr in the Treatment of Pick Complex/Frontotemporal Dementia Completed NCT00416169 Phase 2 galantamine hydrobromide
27 Efficacy and Tolerability of Memantine in Frontotemporal Dementia (FTD) Patients Completed NCT00200538 Phase 2 memantine
28 Imaging Characteristics of Florbetapir 18F in Patients With Frontotemporal Dementia, Alzheimer's Disease and Normal Controls. Completed NCT01890343 Phase 2 florbetapir 18F;18F-FDG
29 Davunetide (AL-108) in Predicted Tauopathies - Pilot Study Completed NCT01056965 Phase 2 davunetide (AL-108, NAP);Placebo nasal spray
30 Double Blind Trial of DC Polarization in FTD Completed NCT00117858 Phase 2
31 Unrelated Hematopoietic Stem Cell Transplantation(HSCT) for Genetic Diseases of Blood Cells Completed NCT00730314 Phase 1, Phase 2
32 Miglustat in Niemann-Pick Type C Disease Completed NCT00517153 Phase 2 miglustat
33 Intranasal Oxytocin for Frontotemporal Dementia Recruiting NCT03260920 Phase 2 Syntocinon
34 Low-Dose Lithium for the Treatment of Behavioral Symptoms in Frontotemporal Dementia Recruiting NCT02862210 Phase 2 Lithium Carbonate;Placebo
35 Study of IV VTS-270 for Infantile Liver Disease Associated With Niemann-Pick Disease, Type C Recruiting NCT03471143 Phase 1, Phase 2 2-Hydroxypropyl-Beta-Cyclodextrin
36 Impact of Emotional Mimicry and Oxytocin on Frontotemporal Dementia Recruiting NCT01937013 Phase 2 Intranasal oxytocin;Saline Nasal Mist
37 Study of Pharmacokinetics and Preliminary Efficacy in Patients With Niemann-Pick C1 Recruiting NCT02912793 Phase 1, Phase 2 Hydroxypropyl-beta-cyclodextrin
38 Study of Lithium Carbonate to Treat Niemann-Pick Type C1 Disease Recruiting NCT03201627 Phase 1, Phase 2 Lithium Carbonate
39 Safety, Tolerability, PK, and Efficacy Evaluation of Repeat Ascending Doses of Olipudase Alfa in Pediatric Patients <18 Years of Age With Acid Sphingomyelinase Deficiency Recruiting NCT02292654 Phase 1, Phase 2 Olipudase alfa
40 Phase I/II Pilot Study of Mixed Chimerism to Treat Inherited Metabolic Disorders Active, not recruiting NCT01372228 Phase 1, Phase 2
41 F 18 T807 Tau PET Imaging in Familial Amyotrophic Lateral Sclerosis Active, not recruiting NCT02414230 Phase 2 Drug: F 18 T807
42 Clinical Trial in Infants With Rapidly Progressive Lysosomal Acid Lipase Deficiency Active, not recruiting NCT02193867 Phase 2 sebelipase alfa
43 F 18 T807 Tau PET Imaging of Progressive Posterior Cortical Dysfunction (IND 123119, Protocol E) Enrolling by invitation NCT02414282 Phase 2 F 18 T807
44 Tau PET Imaging With 18F-AV-1451 in Subjects With MAPT Mutations Enrolling by invitation NCT02676843 Phase 2 18F-AV-1451
45 A Long-Term Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency Enrolling by invitation NCT02004704 Phase 2 GZ402665
46 Hematopoietic Stem Cell Transplantation (HCT) for Inborn Errors of Metabolism Terminated NCT00668564 Phase 2 Cyclophosphamide;Campath-1H;Busulfan
47 F 18 T807 Tau PET Imaging of Frontotemporal Dementia (FTD) Withdrawn NCT02707978 Phase 2 F 18 T807
48 Far Infrared Irradiation for the Management, Control and Treatment of Frontotemporal Dementia Unknown status NCT00674960 Phase 1
49 Far Infrared Radiation Treatment of Dementia and Other Mental Illness Unknown status NCT00574054 Phase 1
50 Safety Study of Intranasal Oxytocin in Frontotemporal Dementia Completed NCT01386333 Phase 1 oxytocin;Saline Nasal Mist

Search NIH Clinical Center for Grn-Related Frontotemporal Dementia

Genetic Tests for Grn-Related Frontotemporal Dementia

Anatomical Context for Grn-Related Frontotemporal Dementia

MalaCards organs/tissues related to Grn-Related Frontotemporal Dementia:

41
Brain, Testes, Prefrontal Cortex, Liver, Cortex, Bone, Eye

Publications for Grn-Related Frontotemporal Dementia

Articles related to Grn-Related Frontotemporal Dementia:

# Title Authors Year
1
GRN-Related Frontotemporal Dementia ( 20301545 )
1993

Variations for Grn-Related Frontotemporal Dementia

Expression for Grn-Related Frontotemporal Dementia

LifeMap Discovery
Genes differentially expressed in tissues of Grn-Related Frontotemporal Dementia patients vs. healthy controls: 35
# Gene Description Tissue Up/Dn Fold Change (log2) P value
1 NEFL neurofilament, light polypeptide NULL - 3.74 0.002
2 VSNL1 visinin-like 1 NULL - 3.71 0.005
3 PCDH8 protocadherin 8 NULL - 3.66 0.000
4 PCSK1 proprotein convertase subtilisin/kexin type 1 NULL - 3.65 0.002
5 ATP1A3 ATPase, Na+/K+ transporting, alpha 3 polypeptide NULL - 3.24 0.002
6 RGS4 regulator of G-protein signaling 4 NULL - 3.21 0.001
7 SYT1 synaptotagmin I NULL - 3.20 0.005
8 STMN2 stathmin 2 NULL - 3.07 0.001
Search GEO for disease gene expression data for Grn-Related Frontotemporal Dementia.

Pathways for Grn-Related Frontotemporal Dementia

GO Terms for Grn-Related Frontotemporal Dementia

Sources for Grn-Related Frontotemporal Dementia

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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