FTLD
MCID: GRN060
MIFTS: 20

Grn-Related Frontotemporal Lobar Degeneration (FTLD)

Categories: Mental diseases

Aliases & Classifications for Grn-Related Frontotemporal Lobar Degeneration

MalaCards integrated aliases for Grn-Related Frontotemporal Lobar Degeneration:

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

Classifications:



Summaries for Grn-Related Frontotemporal Lobar Degeneration

Genetics Home Reference : 25 GRN-related frontotemporal lobar degeneration 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 7 to 13 years after the appearance of symptoms. However, symptoms can begin as early as a person's thirties or as late as a person's eighties. The features of this condition vary significantly, even among affected members of the same family. GRN Behavioral changes are the most common early signs of GRN-related frontotemporal lobar degeneration. These include marked changes in personality, judgment, and insight. It may become difficult for affected individuals to interact with others in a socially appropriate manner. Affected people may also become easily distracted and unable to complete tasks. They increasingly require help with personal care and other activities of daily living. GRN Many people with GRN-related frontotemporal lobar degeneration develop progressive problems with speech and language (aphasia). Affected individuals may have trouble speaking, remembering words and names (dysnomia), and understanding speech. Over time, they may completely lose the ability to communicate (mutism). People with this condition also experience a decline in intellectual function (dementia). GRN Some people with GRN-related frontotemporal lobar degeneration also develop movement disorders, such as parkinsonism and corticobasal syndrome. The signs and symptoms of these disorders include tremors, muscle stiffness (rigidity), unusually slow movement (bradykinesia), walking problems (gait disturbance), involuntary muscle spasms (myoclonus), uncontrolled muscle tensing (dystonia), and an inability to carry out purposeful movements (apraxia). GRN

MalaCards based summary : Grn-Related Frontotemporal Lobar Degeneration, also known as hereditary dysphasic disinhibition dementia, is related to frontotemporal dementia and frontotemporal dementia and/or amyotrophic lateral sclerosis 1. An important gene associated with Grn-Related Frontotemporal Lobar Degeneration is GRN (Granulin Precursor). The drugs Memantine and Citalopram have been mentioned in the context of this disorder. Affiliated tissues include brain.

Related Diseases for Grn-Related Frontotemporal Lobar Degeneration

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

(show top 50) (show all 151)
# Related Disease Score Top Affiliating Genes
1 frontotemporal dementia 12.7
2 frontotemporal dementia and/or amyotrophic lateral sclerosis 1 12.4
3 frontotemporal lobar degeneration with tdp43 inclusions, grn-related 12.2
4 amyotrophic lateral sclerosis 10 with or without frontotemporal dementia 12.1
5 semantic dementia 12.0
6 progressive non-fluent aphasia 11.9
7 pick disease of brain 11.9
8 perry syndrome 11.5
9 aphasia 11.2
10 amyotrophic lateral sclerosis 1 11.2
11 lateral sclerosis 11.2
12 alzheimer disease 11.1
13 motor neuron disease 11.0
14 corticobasal degeneration 11.0
15 supranuclear palsy, progressive, 1 11.0
16 dementia 10.8
17 grn frontotemporal dementia 10.7
18 apraxia 10.7
19 frontotemporal dementia, chromosome 3-linked 10.6
20 speech and communication disorders 10.5
21 multiple system atrophy 1 10.5
22 cerebral atrophy 10.5
23 tardbp-related amyotrophic lateral sclerosis 10.5
24 primary lateral sclerosis, adult, 1 10.4
25 paget's disease of bone 10.4
26 neuronal ceroid lipofuscinosis 10.4
27 major affective disorder 8 10.3
28 major affective disorder 9 10.3
29 multisystem proteinopathy 10.3
30 amnestic disorder 10.3
31 pseudobulbar palsy 10.3
32 bipolar disorder 10.3
33 myopathy 10.3
34 nominal aphasia 10.3
35 neuroblastoma 10.3
36 vascular dementia 10.3
37 c9orf72-related amyotrophic lateral sclerosis and frontotemporal dementia 10.3
38 autonomic dysfunction 10.3
39 cerebral amyloid angiopathy, cst3-related 10.2
40 creutzfeldt-jakob disease 10.2
41 huntington disease 10.2
42 parkinson disease, late-onset 10.2
43 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
44 frontotemporal dementia and/or amyotrophic lateral sclerosis 3 10.2
45 hydrocephalus 10.2
46 dyscalculia 10.2
47 quadriplegia 10.2
48 mutism 10.2
49 movement disease 10.2
50 prion disease 10.2

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



Diseases related to Grn-Related Frontotemporal Lobar Degeneration

Symptoms & Phenotypes for Grn-Related Frontotemporal Lobar Degeneration

Drugs & Therapeutics for Grn-Related Frontotemporal Lobar Degeneration

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

(show all 37)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Memantine Approved, Investigational Phase 4 19982-08-2 4054
2
Citalopram Approved Phase 4 59729-33-8 2771
3
Acetaminophen Approved Phase 4 103-90-2 1983
4
Buprenorphine Approved, Illicit, Investigational, Vet_approved Phase 4 52485-79-7 40400 644073
5 Dopamine Agents Phase 4
6 Neurotransmitter Agents Phase 4
7 Excitatory Amino Acid Antagonists Phase 4
8 Antiparkinson Agents Phase 4
9 Antidepressive Agents Phase 4
10 Psychotropic Drugs Phase 4
11 Serotonin Uptake Inhibitors Phase 4
12 Analgesics, Non-Narcotic Phase 4
13 Narcotics Phase 4
14 Analgesics Phase 4
15 Analgesics, Opioid Phase 4
16 Narcotic Antagonists Phase 4
17 Antipyretics Phase 4
18
Serotonin Investigational, Nutraceutical Phase 4 50-67-9 5202
19
Galantamine Approved Phase 2 357-70-0 9651
20 Catechol Phase 2
21 Cholinesterase Inhibitors Phase 2
22 Cholinergic Agents Phase 2
23
Tyrosine Approved, Investigational, Nutraceutical Phase 1 60-18-4 6057
24
Corticosterone Experimental Phase 1 50-22-6 5753
25 Fluorodeoxyglucose F18 Phase 1
26 Deoxyglucose Phase 1
27 Radiopharmaceuticals Phase 1
28 Pharmaceutical Solutions Phase 1
29 Tin Fluorides Phase 1
30 Immunoglobulins Phase 1
31 Antibodies Phase 1
32 Antibodies, Monoclonal Phase 1
33
Dopamine Approved 51-61-6, 62-31-7 681
34 Central Nervous System Stimulants
35 Antipsychotic Agents
36 polysaccharide-K
37 Dopamine agonists

Interventional clinical trials:

(show all 50)
# Name Status NCT ID Phase Drugs
1 A 52 Week Open Label Trial of Memantine for Frontotemporal Lobar Degeneration Completed NCT00187525 Phase 4 Memantine
2 Serotonergic Function and Behavioural and Psychological Symptoms of Frontotemporal Dementia Completed NCT00376051 Phase 4 Citalopram
3 Efficacy of Pain Treatment on Depression in Patients With Dementia. A Randomized Clinical Trial. Completed NCT02267057 Phase 4 Paracetamol;Buprenorphine;Paracetamol placebo;Buprenorphine placebo
4 Tau PET Imaging With 18F-AV-1451 in Subjects With MAPT Mutations Completed NCT02676843 Phase 2 18F-AV-1451
5 Investigation of the Dopamine System in Frontotemporal Dementia Completed NCT00604591 Phase 2 Tolcapone;Placebo
6 An Open Pilot Study to Evaluate the Safety and Efficacy of Galantamine in the Treatment of Pick's Disease/Frontotemporal Dementia /Pick Complex Completed NCT00416169 Phase 2 galantamine hydrobromide
7 A Phase 1/2 Ascending Dose Study to Evaluate the Safety and Effects on Progranulin Levels of PR006A in Patients With Fronto-Temporal Dementia With Progranulin Mutations (FTD-GRN) Not yet recruiting NCT04408625 Phase 1, Phase 2
8 A Noval Tau Tracer ([18F]PM-PBB3) in Young Onset Dementia: Clinical and Neuroimaging Study Not yet recruiting NCT04248270 Phase 1, Phase 2 18F-PM-PBB3
9 A 12 Week Randomized, Double Blind, Placebo-Controlled Pilot Study of Davunetide (NAP, AL-108) in Predicted Tauopathies Completed NCT01056965 Phase 1 davunetide (AL-108, NAP);Placebo nasal spray
10 PiB PET Scanning in Speech and Language Based Dementias Completed NCT01623284 Phase 1 C-11 PiB;F-18 FDG
11 A First in Human Phase 1 Study in Healthy Volunteers and in Participants With Frontotemporal Dementia (FTD) With Granulin Mutation Recruiting NCT03636204 Phase 1
12 Alzheimer's PET Imaging in Racially/Ethnically Diverse Adults Recruiting NCT03706261 Phase 1 18F-MK-6240;18F-Florbetaben
13 Open Label Study for the Use of Tyrosine Kinase Inhibitors for Treatment of Cognitive Decline Due to Degenerative Dementias Enrolling by invitation NCT02921477 Phase 1 bosutinib
14 A Phase 1b, Randomized, Double-Blind, Placebo-Controlled, Parallel Cohort Safety, Tolerability, Pharmacokinetics, Pharmacodynamics and Preliminary Efficacy Study of Intravenously Infused BIIB092 in Patients With Four Different Primary Tauopathy Syndromes Terminated NCT03658135 Phase 1 BIIB092
15 Identification of New Genes Causing Frontotemporal Lobar Degeneration by Whole Exome Sequencing and Characterization of the Associated Phenotypes Unknown status NCT02363062
16 Social Cognition in Ageing and in Frontotemporal Lobar Degeneration (Frontotemporal Dementia and Semantic Dementia): a Cognitive and Neuroimaging Study Unknown status NCT01962064
17 Predict to Prevent Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis Unknown status NCT02590276
18 Patients With Alzheimer's Disease or Related Youth Disease Unknown status NCT03508024
19 Innovative Biomarkers in Alzheimer's Disease and Frontotemporal Dementia (FTD): Preventative and Personalized Unknown status NCT01403519
20 Psycho-behavioral Disorders in Frontotemporal Lobar Degeneration: Validation of a Quantification and Follow-up Scale Completed NCT02889601
21 Neuropsychological and Anatomical Study of Concept Formation in Frontal Patients Completed NCT01100281
22 A Non-Blinded, Non-Significant Risk Study With a Non-Invasive, Passive Pressure Wave Method of Diagnosing Brain Pathologies to Develop a Diagnostic Algorithm for Alzheimer Disease and Other Dementias. Completed NCT02333942
23 The Ontario Neurodegenerative Disease Research Initiative Completed NCT04104373
24 University of California, San Francisco (UCSF) and University of Nebraska Medical Center (UNMC) Care Ecosystem Completed NCT02213458
25 Treatment Study for Frontotemporal Dementia Completed NCT00088751
26 Rehabilitative Trial for the Recovery of Neurophysiological Parameters in Progranulin Mutation Carriers Through the Use of Transcranial Direct Current Stimulation (tDCS) Completed NCT02999282
27 PET Evaluation of Brain Peripheral Benzodiazepine Receptors Using [11C]PBR28 in Neurological Disorders Completed NCT00613119
28 Assessment of Social-emotional Functioning in Stroke, Frontotemporal Dementia, Alzheimer and Parkinson Diseases Completed NCT01339130
29 Measurement of P-Glycoprotein Function in Alzheimer Disease, Parkinson Disease, and Frontotemporal Dementia Using Positron Emission Tomography Completed NCT00677885
30 Pilot Study of EEG and Cerebral Blood Flow Biofeedback Training in Remediating Cognitive and Behavioral Deficits in Adults With a Dementing Illness. Completed NCT01168466
31 ARTFL LEFFTDS Longitudinal Frontotemporal Lobar Degeneration (ALLFTD) Recruiting NCT04363684
32 Multimodal Assessment For Predicting Specific Pathological Substrate in Frontotemporal Lobar Degeneration Recruiting NCT02964637
33 Assessing Changes in Social Cognition and Personality in Patients With Frontotemporal Lobar Degeneration, Alzheimer's Disease and Parkinson's Disease and Their Effect on the Patient-caregiver Relationship Recruiting NCT02964611
34 Multidisciplinary and Personalized Care of Behavioral Disorders in Frontotemporal Lobar Degeneration. Recruiting NCT03606798
35 Rare Diseases Clinical Research Network Advancing Research and Treatment for Frontotemporal Lobar Degeneration [ARTFL]: Research Projects 1 & 2 Recruiting NCT02365922
36 Natural History Characterization in Symptomatic and Asymptomatic Progranuline Gene Mutation Carriers Recruiting NCT04014673
37 Hippocampal Sclerosis and Amnesia Not Due to Alzheimer's Disease Recruiting NCT02576821
38 Investigating Complex Neurodegenerative Disorders Related to Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Recruiting NCT03225144
39 Language in Primary Progressive Aphasia Recruiting NCT00537004
40 Assessment of Apathy in a Real-life Situation, With a Video and Sensors-based System in Healthy Subject and Patient With Cerebral Disease Recruiting NCT03272230
41 Self-consciousness in Dementia Recruiting NCT01782248
42 A Multi-centre Proof-of-performance Clinical Study to Validate Blood-based Biomarker Candidates for the Diagnosis of Alzheimer's Disease Recruiting NCT03030586
43 Smell, Voice and Nasal Swabs as Markers for Neuro-degenerative Disorders Recruiting NCT03299062
44 Investigation of the Therapeutic Value of Transcranial Stimulation With Direct Current on Language Disorders in Semantic Dementia Recruiting NCT03481933
45 Communication Bridge Speech Therapy Research Study: Using Internet-Based Speech Therapy to Improve Quality of Life and Access to Care Active, not recruiting NCT02439853
46 Care Ecosystem: Navigating Patients and Families Through Stages of Care, Extension Trial Active, not recruiting NCT04287738
47 Natural History and Biomarkers of C9ORF72 Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Active, not recruiting NCT01925196
48 Imaging Tau Deposition in the Brain of Elderly Subjects Enrolling by invitation NCT02958670
49 A Randomized, Double-blinded, Sham-controlled Cross-over Study of Theta-burst Transcranial Magnetic Stimulation in Nonfluent/Agrammatic Variant Primary Progressive Aphasia Not yet recruiting NCT03153540
50 Biomarkers in Neurodegenerative Diseases Withdrawn NCT04055532

Search NIH Clinical Center for Grn-Related Frontotemporal Lobar Degeneration

Genetic Tests for Grn-Related Frontotemporal Lobar Degeneration

Anatomical Context for Grn-Related Frontotemporal Lobar Degeneration

MalaCards organs/tissues related to Grn-Related Frontotemporal Lobar Degeneration:

40
Brain

Publications for Grn-Related Frontotemporal Lobar Degeneration

Articles related to Grn-Related Frontotemporal Lobar Degeneration:

# Title Authors PMID Year
1
GFRA2 in GRN-related frontotemporal lobar degeneration. 61
29724593 2018
2
Novel GRN Mutations in Patients with Corticobasal Syndrome. 61
26961809 2016
3
Role of progranulin as a biomarker for Alzheimer's disease. 61
20387302 2010
4
Corticobasal syndrome associated with the A9D Progranulin mutation. 61
17917583 2007
5
Neuropathologic heterogeneity in HDDD1: a familial frontotemporal lobar degeneration with ubiquitin-positive inclusions and progranulin mutation. 61
17334266 2007
6
HDDD2 is a familial frontotemporal lobar degeneration with ubiquitin-positive, tau-negative inclusions caused by a missense mutation in the signal peptide of progranulin. 61
16983685 2006
7
Loss of brain tau defines novel sporadic and familial tauopathies with frontotemporal dementia. 61
11220736 2001
8
Hereditary dysphasic disinhibition dementia: a frontotemporal dementia linked to 17q21-22. 61
9633693 1998

Variations for Grn-Related Frontotemporal Lobar Degeneration

Expression for Grn-Related Frontotemporal Lobar Degeneration

Search GEO for disease gene expression data for Grn-Related Frontotemporal Lobar Degeneration.

Pathways for Grn-Related Frontotemporal Lobar Degeneration

GO Terms for Grn-Related Frontotemporal Lobar Degeneration

Sources for Grn-Related Frontotemporal Lobar Degeneration

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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