DDPAC
MCID: FRN030
MIFTS: 29

Frontotemporal Dementia with Parkinsonism-17 (DDPAC) malady

Categories: Genetic diseases, Mental diseases

Aliases & Classifications for Frontotemporal Dementia with Parkinsonism-17

Aliases & Descriptions for Frontotemporal Dementia with Parkinsonism-17:

Name: Frontotemporal Dementia with Parkinsonism-17 24 25 69
Pallidopontonigral Degeneration 24 69
Wilhelmsen-Lynch Disease 24 25
Ftdp-17 24 25
Disinhibition-Dementia-Parkinsonism-Amyotrophy Complex 24
Disinhibition-Dementia-Parkinsonism-Amytrophy Complex 25
Pallido-Ponto-Nigral Degeneration 24
Hereditary Pick's Disease 24
Frontotemporal Dementia 69
Familial Pick's Disease 25
Ddpac 25

Classifications:



Summaries for Frontotemporal Dementia with Parkinsonism-17

Genetics Home Reference : 25 Frontotemporal dementia with parkinsonism-17 (FTDP-17) is a brain disorder. It is part of a group of conditions, called frontotemporal dementia or frontotemporal degeneration, that are characterized by a loss of nerve cells (neurons) in areas of the brain called the frontal and temporal lobes. Over time, a loss of these cells can affect personality, behavior, language, and movement.

MalaCards based summary : Frontotemporal Dementia with Parkinsonism-17, also known as pallidopontonigral degeneration, is related to dementia, frontotemporal and grn-related frontotemporal dementia, and has symptoms including personality changes An important gene associated with Frontotemporal Dementia with Parkinsonism-17 is MAPT (Microtubule Associated Protein Tau), and among its related pathways/superpathways are Neuroscience and Alzheimers Disease Pathway. The drugs Dopamine and Memantine have been mentioned in the context of this disorder. Affiliated tissues include temporal lobe and brain, and related phenotype is vision/eye.

Related Diseases for Frontotemporal Dementia with Parkinsonism-17

Diseases related to Frontotemporal Dementia with Parkinsonism-17 via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 45)
id Related Disease Score Top Affiliating Genes
1 dementia, frontotemporal 11.2
2 grn-related frontotemporal dementia 10.8
3 dementia 10.4
4 mcleod neuroacanthocytosis syndrome 10.1 MAPT PSEN1
5 intracranial structure hemangioma 10.0 MAPT PSEN1
6 meckel syndrome 10 10.0 MAPT PSEN1
7 social emotional agnosia 10.0 GRN PSEN1
8 color agnosia 10.0 GRN PSEN1
9 pancreatitis 10.0 GRN PSEN1
10 autoimmune disease of blood 10.0 GRN PSEN1
11 encephalitozoonosis 10.0 GRN PSEN1
12 contagious pustular dermatitis 10.0 MAPT PSEN1
13 acquired metabolic disease 10.0 GRN PSEN1
14 hyperglycemia 9.9 GRN PSEN1
15 protein r deficiency 9.9 GRN MAPT
16 acne inversa, familial, 3 9.9 MAPT PSEN1
17 pediatric mesenchymal chondrosarcoma 9.9 GRN PSEN1
18 femoral neuropathy 9.9 GRN MAPT
19 pancreatic cancer/melanoma syndrome 9.9 GRN PSEN1
20 epithelioid type angiomyolipoma 9.9 MAPT PSEN1
21 mitochondrial dna depletion syndrome 3 9.9 GRN MAPT
22 meier-gorlin syndrome 5 9.9 GRN MAPT
23 autotopagnosia 9.9 GRN MAPT
24 autism spectrum disorder 9.8 GRN PSEN1
25 alzheimer disease 9.8
26 corticobasal degeneration 9.8
27 gata1-related thrombocytopenia 9.8 GRN MAPT PSEN1
28 hereditary paraganglioma-pheochromocytoma syndromes 9.8 GRN MAPT PSEN1
29 cerebroretinal vasculopathy 9.8 GRN MAPT PSEN1
30 severe congenital nemaline myopathy 9.8 GRN MAPT PSEN1
31 andersen syndrome 9.8 GRN MAPT PSEN1
32 acrofrontofacionasal dysostosis 9.8 GRN MAPT PSEN1
33 postauricular lymphadenitis 9.8 GRN MAPT PSEN1
34 adult mesenchymal chondrosarcoma 9.8 GRN MAPT PSEN1
35 autoimmune disease of urogenital tract 9.7 GRN MAPT PSEN1
36 pancreatic solid pseudopapillary carcinoma 9.7 GRN MAPT PSEN1
37 coenzyme q10 deficiency, primary, 1 9.7 GRN MAPT PSEN1
38 angina pectoris 9.7 GRN MAPT PSEN1
39 cardiomyopathy, dilated, 1u 9.7 GRN MAPT PSEN1
40 personality disorder 9.7 GRN MAPT PSEN1
41 eumycotic mycetoma 9.7 GRN MAPT PSEN1
42 substance-induced psychosis 9.7 GRN MAPT PSEN1
43 long qt syndrome 5 9.7 GRN MAPT PSEN1
44 mucopolysaccharidosis iv 9.7 GRN MAPT
45 spondyloarthropathy 1 9.6 GRN MAPT PSEN1

Graphical network of the top 20 diseases related to Frontotemporal Dementia with Parkinsonism-17:



Diseases related to Frontotemporal Dementia with Parkinsonism-17

Symptoms & Phenotypes for Frontotemporal Dementia with Parkinsonism-17

UMLS symptoms related to Frontotemporal Dementia with Parkinsonism-17:


personality changes

MGI Mouse Phenotypes related to Frontotemporal Dementia with Parkinsonism-17:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 vision/eye MP:0005391 8.8 GRN MAPT PSEN1

Drugs & Therapeutics for Frontotemporal Dementia with Parkinsonism-17

Drugs for Frontotemporal Dementia with Parkinsonism-17 (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 99)
id 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
Citalopram Approved Phase 4 59729-33-8 2771
4
Amantadine Approved Phase 4 768-94-5 2130
5 Fluorodeoxyglucose F18 Phase 4,Phase 2,Phase 1
6
Corticosterone Phase 4,Phase 1,Early Phase 1 50-22-6 5753
7
Serotonin Phase 4 50-67-9 5202
8 Serotonin Agents Phase 4
9 Serotonin Uptake Inhibitors Phase 4
10 Neurotransmitter Agents Phase 4,Phase 3,Phase 2
11 Neurotransmitter Uptake Inhibitors Phase 4
12 Peripheral Nervous System Agents Phase 4,Phase 2
13 Dopamine Agents Phase 4,Phase 3,Phase 2,Phase 1
14 Excitatory Amino Acid Antagonists Phase 4,Phase 3,Phase 2
15 Excitatory Amino Acids Phase 4,Phase 3,Phase 2
16 Anti-Infective Agents Phase 4,Phase 3,Phase 1,Phase 2
17 Antidepressive Agents Phase 4,Phase 2
18 Antidepressive Agents, Second-Generation Phase 4
19 Psychotropic Drugs Phase 4,Phase 2
20 Antiviral Agents Phase 4,Phase 3,Phase 1,Phase 2
21 Antiparkinson Agents Phase 4,Phase 3,Phase 2
22 Pharmaceutical Solutions Phase 4,Phase 2,Phase 3,Phase 1
23
Iodine Phase 4 7553-56-2 807
24 cadexomer iodine Phase 4
25 Anti-Inflammatory Agents Phase 4
26 Analgesics Phase 4
27 Analgesics, Non-Narcotic Phase 4
28 Calamus Nutraceutical Phase 4
29
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
30
Busulfan Approved, Investigational Phase 2, Phase 3 55-98-1 2478
31
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 5755
32
Miglustat Approved Phase 3,Phase 2,Phase 1 72599-27-0 51634
33
Benzocaine Approved Phase 3 1994-09-7, 94-09-7 2337
34
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 6741
35 tannic acid Approved, Nutraceutical Phase 3
36
1-Deoxynojirimycin Experimental Phase 3,Phase 2 19130-96-2 1374
37 Prednisolone acetate Phase 2, Phase 3
38 Alkylating Agents Phase 2, Phase 3
39 Glycoside Hydrolase Inhibitors Phase 3,Phase 2,Phase 1
40 Hypoglycemic Agents Phase 3,Phase 2,Phase 1
41 Anti-HIV Agents Phase 3,Phase 2,Phase 1
42 Prednisolone hemisuccinate Phase 2, Phase 3
43 Prednisolone phosphate Phase 2, Phase 3
44 Immunosuppressive Agents Phase 2, Phase 3
45 Antilymphocyte Serum Phase 2, Phase 3
46 Anti-Retroviral Agents Phase 3,Phase 2,Phase 1
47 Methylprednisolone acetate Phase 2, Phase 3
48 Methylprednisolone Hemisuccinate Phase 2, Phase 3
49 Antirheumatic Agents Phase 2, Phase 3
50 Antineoplastic Agents, Alkylating Phase 2, Phase 3

Interventional clinical trials:

(show top 50) (show all 132)
id Name Status NCT ID Phase
1 Memantine (10mg BID) for the Frontal and Temporal Subtypes of Frontotemporal Dementia Completed NCT00545974 Phase 4
2 Serotonergic Function and Behavioural and Psychological Symptoms of Frontotemporal Dementia Completed NCT00376051 Phase 4
3 Imaging of Brain Amyloid Plaques in the Aging Population Enrolling by invitation NCT00950430 Phase 4
4 DaTSCAN Imaging in Aging and Neurodegenerative Disease Enrolling by invitation NCT01453127 Phase 4
5 Amantadine for the Treatment of Behavioral Disturbance in Frontotemporal Dementia (FTD) Terminated NCT00127114 Phase 4
6 Safety and Efficacy Study Evaluating TRx0237 in Subjects With Behavioral Variant Frontotemporal Dementia (bvFTD) Completed NCT01626378 Phase 3
7 Open Label Pilot Study of the Effects of Memantine on FDG-PET in Frontotemporal Dementia Completed NCT00594737 Phase 3
8 Application of Miglustat in Patients With Niemann-Pick Type C Completed NCT01760564 Phase 3
9 Stem Cell Transplant for Inborn Errors of Metabolism Completed NCT00176904 Phase 2, Phase 3
10 Study of VTS-270 (2-hydroxypropyl-β-cyclodextrin) to Treat Niemann-Pick Type C1 (NPC1) Disease Recruiting NCT02534844 Phase 2, Phase 3
11 Efficacy, Safety, Pharmacodynamic, and Pharmacokinetics Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency Recruiting NCT02004691 Phase 2, Phase 3
12 Arimoclomol Prospective Study in Patients Diagnosed With NiemannPick Disease Type C Active, not recruiting NCT02612129 Phase 2, Phase 3
13 SurVival of Lysosomal Acid Lipase Deficiency (LAL-D) Infants Treated With SebelipAse aLfa Active, not recruiting NCT01371825 Phase 2, Phase 3
14 Open-Label Study of TRx0237 in Subjects With Alzheimer's Disease or Behavioral Variant Frontotemporal Dementia (bvFTD) Enrolling by invitation NCT02245568 Phase 3
15 ALD-101 Adjuvant Therapy of Unrelated Umbilical Cord Blood Transfusion (UCBT) in Patients With Inherited Metabolic Diseases Terminated NCT00654433 Phase 3
16 Children With Lysosomal Acid Lipase Deficiency Who Previously Received Treatment With SBC-102 Terminated NCT01473875 Phase 2, Phase 3
17 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
18 Phase 1/2 Study of Vorinostat Therapy in Niemann-Pick Disease, Type C1 Completed NCT02124083 Phase 1, Phase 2
19 A Pilot Study to Explore the Safety and Tolerability of Galantamine HBr in the Treatment of Pick Complex/Frontotemporal Dementia Completed NCT00416169 Phase 2
20 Biomarker Validation for Niemann-Pick Disease, Type C: Safety and Efficacy of N-Acetyl Cysteine Completed NCT00975689 Phase 1, Phase 2
21 Efficacy and Tolerability of Memantine in Frontotemporal Dementia (FTD) Patients Completed NCT00200538 Phase 2
22 Imaging Characteristics of Florbetapir 18F in Patients With Frontotemporal Dementia, Alzheimer's Disease and Normal Controls. Completed NCT01890343 Phase 2
23 Electroencephalography (EEG) Biofeedback Training to Improve Executive Functioning and Memory in Adults With a Dementing Illness Completed NCT01168466 Phase 2
24 Double Blind Trial of DC Polarization in FTD Completed NCT00117858 Phase 2
25 Unrelated Hematopoietic Stem Cell Transplantation(HSCT) for Genetic Diseases of Blood Cells Completed NCT00730314 Phase 1, Phase 2
26 Miglustat in Niemann-Pick Type C Disease Completed NCT00517153 Phase 2
27 Low-Dose Lithium for the Treatment of Behavioral Symptoms in Frontotemporal Dementia Recruiting NCT02862210 Phase 2
28 Impact of Emotional Mimicry and Oxytocin on Frontotemporal Dementia Recruiting NCT01937013 Phase 2
29 Study of Pharmacokinetics and Preliminary Efficacy in Patients With Niemann-Pick C1 Recruiting NCT02912793 Phase 1, Phase 2
30 F 18 T807 Tau PET Imaging in Familial Amyotrophic Lateral Sclerosis Recruiting NCT02414230 Phase 2
31 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
32 Effects of Tolcapone on Frontotemporal Dementia Active, not recruiting NCT00604591 Phase 2
33 Davunetide (AL-108) in Predicted Tauopathies - Pilot Study Active, not recruiting NCT01056965 Phase 2
34 Phase I/II Pilot Study of Mixed Chimerism to Treat Inherited Metabolic Disorders Active, not recruiting NCT01372228 Phase 1, Phase 2
35 Clinical Trial in Infants With Rapidly Progressive Lysosomal Acid Lipase Deficiency Active, not recruiting NCT02193867 Phase 2
36 Tau PET Imaging With 18F-AV-1451 in Subjects With MAPT Mutations Enrolling by invitation NCT02676843 Phase 2
37 A Long-Term Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency Enrolling by invitation NCT02004704 Phase 2
38 F 18 T807 Tau PET Imaging of Frontotemporal Dementia (FTD) Not yet recruiting NCT02707978 Phase 2
39 Hematopoietic Stem Cell Transplantation (HCT) for Inborn Errors of Metabolism Terminated NCT00668564 Phase 2
40 Far Infrared Irradiation for the Management, Control and Treatment of Frontotemporal Dementia Unknown status NCT00674960 Phase 1
41 Clinical and Genetic Study of Neurodegenerative Disorders With Cognitive Impairment Unknown status NCT00149175 Phase 1
42 Far Infrared Radiation Treatment of Dementia and Other Mental Illness Unknown status NCT00574054 Phase 1
43 Safety Study of Intranasal Oxytocin in Frontotemporal Dementia Completed NCT01386333 Phase 1
44 Direct Current Brain Polarization in Frontotemporal Dementia Completed NCT00077896 Phase 1
45 Hydroxypropyl Beta Cyclodextrin for Niemann-Pick Type C1 Disease Completed NCT01747135 Phase 1
46 Saccadic Eye Movements in Patients With Niemann-Pick Type C Disease Completed NCT00316498 Phase 1
47 Amyloid-related Imaging Abnormalities (Microbleeds) in Atypical AD Completed NCT01723553 Phase 1
48 Dose Finding Study of Nimodipine for the Treatment of Progranulin Insufficiency From GRN Gene Mutations Completed NCT01835665 Phase 1
49 Evaluation of [18F]MNI-777 PET as a Marker of Tau Pathology in Subjects With Tauopathies Compared to Healthy Subjects Completed NCT02103894 Phase 1
50 PiB PET Scanning in Speech and Language Based Dementias Completed NCT01623284 Phase 1

Search NIH Clinical Center for Frontotemporal Dementia with Parkinsonism-17

Genetic Tests for Frontotemporal Dementia with Parkinsonism-17

Genetic tests related to Frontotemporal Dementia with Parkinsonism-17:

id Genetic test Affiliating Genes
1 Frontotemporal Dementia with Parkinsonism-17 24 MAPT

Anatomical Context for Frontotemporal Dementia with Parkinsonism-17

MalaCards organs/tissues related to Frontotemporal Dementia with Parkinsonism-17:

39
Temporal Lobe, Brain

Publications for Frontotemporal Dementia with Parkinsonism-17

Variations for Frontotemporal Dementia with Parkinsonism-17

Expression for Frontotemporal Dementia with Parkinsonism-17

Search GEO for disease gene expression data for Frontotemporal Dementia with Parkinsonism-17.

Pathways for Frontotemporal Dementia with Parkinsonism-17

Pathways related to Frontotemporal Dementia with Parkinsonism-17 according to GeneCards Suite gene sharing:

id Super pathways Score Top Affiliating Genes
1 11.47 MAPT PSEN1
2 10.28 MAPT PSEN1

GO Terms for Frontotemporal Dementia with Parkinsonism-17

Cellular components related to Frontotemporal Dementia with Parkinsonism-17 according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 axon GO:0030424 8.96 MAPT PSEN1
2 growth cone GO:0030426 8.62 MAPT PSEN1

Biological processes related to Frontotemporal Dementia with Parkinsonism-17 according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 memory GO:0007613 8.62 MAPT PSEN1

Sources for Frontotemporal Dementia with Parkinsonism-17

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
Content
Loading form....