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familial Danish Dementia

Cerebellar ataxia, cataract, deafness, and dementia or psychosis
Also Known As:
Dementia, familial Danish; Cerebral Amyloid Angiopathy, Itm2b-Related, 2; Familial Danish dementia; Heredopathia ophthalmootoencephalica
Networked: 33 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Related Diseases

1. Dementia (Dementias)
2. Neurodegenerative Diseases (Neurodegenerative Disease)
3. Alzheimer Disease (Alzheimer's Disease)
4. Amyloidosis
5. Cerebral Amyloid Angiopathy (Congophilic Angiopathy)

Experts

1. Ghiso, Jorge: 9 articles (01/2018 - 03/2002)
2. Lashley, Tammaryn: 7 articles (01/2018 - 03/2002)
3. Vidal, Ruben: 6 articles (10/2020 - 03/2002)
4. Revesz, Tamas: 6 articles (01/2018 - 03/2002)
5. Rostagno, Agueda: 6 articles (01/2016 - 03/2002)
6. Frangione, Blas: 6 articles (07/2009 - 03/2002)
7. D'Adamio, Luciano: 5 articles (01/2021 - 11/2010)
8. Holton, Janice L: 5 articles (01/2018 - 03/2002)
9. Plant, Gordon: 5 articles (07/2009 - 03/2002)
10. Cisternas, Pablo: 3 articles (10/2020 - 01/2019)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to familial Danish Dementia:
1. Amyloid (Amyloid Fibrils)IBA
2. Proteins (Proteins, Gene)FDA Link
3. Peptides (Polypeptides)IBA
4. Cystatin CIBA
07/01/2002 - "CAA occurs in several familial conditions, including hereditary cerebral hemorrhage with amyloidosis of Icelandic type caused by deposition of mutant cystatin C, hereditary cerebral hemorrhage with amyloidosis Dutch type and familial AD with deposition of either A beta variants or wild-type A beta, the transthyretin-related meningo-vascular amyloidoses, gelsolin as well as familial prion disease-related CAAs and the recently described BRI2 gene-related CAAs in familial British dementia and familial Danish dementia. "
09/01/2003 - "Other amyloid proteins involved in familial CAAs include 1) the mutant cystatin C (ACys) in hereditary cerebral hemorrhage with amyloidosis of Icelandic type, 2) variant transthyretins (ATTR) in meningo-vascular amyloidoses, 3) mutated gelsolin (AGel) in familial amyloidosis of Finnish type, 4) disease-associated prion protein (PrP(Sc)) in a variant of the Gerstmann-Sträussler-Scheinker syndrome, and 5) ABri and ADan in CAAs observed in the recently described BRI2 gene-related dementias, familial British dementia and familial Danish dementia, respectively. "
07/01/2009 - "These latter proteins include the ABri and ADan subunits in familial British dementia and familial Danish dementia, respectively, which are also known under the umbrella term BRI2 gene-related dementias, variant cystatin C in hereditary cerebral haemorrhage with amyloidosis of Icelandic-type, variant transthyretins in meningo-vascular amyloidosis, disease-associated prion protein (PrP(Sc)) in hereditary prion disease with premature stop codon mutations and mutated gelsolin (AGel) in familial amyloidosis of Finnish type. "
5. GelsolinIBA
07/01/2002 - "CAA occurs in several familial conditions, including hereditary cerebral hemorrhage with amyloidosis of Icelandic type caused by deposition of mutant cystatin C, hereditary cerebral hemorrhage with amyloidosis Dutch type and familial AD with deposition of either A beta variants or wild-type A beta, the transthyretin-related meningo-vascular amyloidoses, gelsolin as well as familial prion disease-related CAAs and the recently described BRI2 gene-related CAAs in familial British dementia and familial Danish dementia. "
09/01/2003 - "Other amyloid proteins involved in familial CAAs include 1) the mutant cystatin C (ACys) in hereditary cerebral hemorrhage with amyloidosis of Icelandic type, 2) variant transthyretins (ATTR) in meningo-vascular amyloidoses, 3) mutated gelsolin (AGel) in familial amyloidosis of Finnish type, 4) disease-associated prion protein (PrP(Sc)) in a variant of the Gerstmann-Sträussler-Scheinker syndrome, and 5) ABri and ADan in CAAs observed in the recently described BRI2 gene-related dementias, familial British dementia and familial Danish dementia, respectively. "
07/01/2009 - "These latter proteins include the ABri and ADan subunits in familial British dementia and familial Danish dementia, respectively, which are also known under the umbrella term BRI2 gene-related dementias, variant cystatin C in hereditary cerebral haemorrhage with amyloidosis of Icelandic-type, variant transthyretins in meningo-vascular amyloidosis, disease-associated prion protein (PrP(Sc)) in hereditary prion disease with premature stop codon mutations and mutated gelsolin (AGel) in familial amyloidosis of Finnish type. "
6. Membrane Proteins (Integral Membrane Proteins)IBA
7. Prion ProteinsIBA
8. Amyloidogenic ProteinsIBA
9. Amyloid Precursor Protein Secretases (beta-Secretase)IBA
10. Nonsense Codon (Nonsense Mutation)IBA

Therapies and Procedures

1. Ligation