Research Models

PSEN1-YAC (line G9)

Synonyms: H163R mutant PS-1 YAC, B6-G9, PS1-YAC

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Species: Mouse
Genes: PSEN1
Modification: PSEN1: Transgenic
Disease Relevance: Alzheimer's Disease
Strain Name: B6.129S4-Tg(PSEN1H163R)G9Btla/J

Summary

Last Updated: 06 Mar 2018

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Research Models

PSEN1-flox

Synonyms: PS1 conditional KO

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Species: Mouse
Genes: PSEN1
Modification: PSEN1: Knock-Out
Disease Relevance: Alzheimer's Disease
Strain Name: B6;129P-Psen1tm1Vln/J

Summary

Last Updated: 20 Apr 2018

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Research Models

APPSwe (line C3-3)

Synonyms: APP(695)Swe

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Species: Mouse
Genes: APP
Modification: APP: Transgenic
Disease Relevance: Alzheimer's Disease
Strain Name: C3B6-Tg(APP695)3Dbo/Mmjax

Summary

Phenotype Characterization

When visualized, these models will distributed over a 18 month timeline demarcated at the following intervals: 1mo, 3mo, 6mo, 9mo, 12mo, 15mo, 18mo+.

Absent

  • Cognitive Impairment

No Data

  • Tangles
  • Neuronal Loss
  • Gliosis
  • Synaptic Loss
  • Changes in LTP/LTD

Plaques

Some plaque formation at advanced age (24-26 months) (Savonenko et al., 2003).

Tangles

No data.

Neuronal Loss

No data.

Gliosis

No data.

Synaptic Loss

No data.

Changes in LTP/LTD

No data.

Cognitive Impairment

Normal reference and working memory up to 12-14 months on congenic background (Savonenko et al., 2003).

Last Updated: 06 Apr 2022

COMMENTS / QUESTIONS

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Further Reading

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Research Models

JNPL3(P301L)

Synonyms: TauP301L-JNPL3

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Species: Mouse
Genes: MAPT
Modification: MAPT: Transgenic
Disease Relevance: Frontotemporal Dementia, Progressive Supranuclear Palsy, Alzheimer's Disease
Strain Name: Tg(Prnp-MAPT*P301L)JNPL3Hlmc

Summary

Phenotype Characterization

When visualized, these models will distributed over a 18 month timeline demarcated at the following intervals: 1mo, 3mo, 6mo, 9mo, 12mo, 15mo, 18mo+.

Absent

  • Plaques

No Data

  • Cognitive Impairment

Plaques

Absent.

Tangles

Neurofibrillary tangles develop in an age and gene-dose dependent manner; as early as 4.5 months in homozygotes and 6.5 months in heterozygotes. Tangles and Pick-body-like neuronal inclusions in the amygdala, septal nuclei, preoptic nuclei, hypothalamus, midbrain, pons, medulla, deep cerebellar nuclei and spinal cord (Lewis et al., 2000).

Neuronal Loss

Neuronal loss, especially in the spinal cord, most prominent in the anterior horn (Lewis et al., 2000).

Gliosis

Astrogliosis (as measured by GFAP reactivity) in brainstem, diencephalon, and basal telencephalon by 10 months (Lewis et al., 2000).

Cognitive Impairment

Unknown.

Last Updated: 02 Feb 2021

COMMENTS / QUESTIONS

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Further Reading

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Research Models

Tg2576/Tau(P301L) (APPSwe-Tau)

Synonyms: APPSwe(2576)/TauJNPL3, TAPP

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Species: Mouse
Genes: APP, MAPT
Modification: APP; MAPT: Transgenic
Disease Relevance: Alzheimer's Disease
Strain Name: Tg(APPSWE)2576Kha; Tg(Prnp-MAPT*P301L)JNPL3Hlmc

Summary

Phenotype Characterization

When visualized, these models will distributed over a 18 month timeline demarcated at the following intervals: 1mo, 3mo, 6mo, 9mo, 12mo, 15mo, 18mo+.

Absent

No Data

  • Synaptic Loss

Plaques

Plaques develop gradually with age. No plaques at 5 months. Very few small plaques at 6 and 7 months. By 9 months plaques scattered throughout the cortex, hippocampus and amygdala, continue to increase at 12 months. Similar distribution as Tg2576.

Tangles

Neurofibrillary tangles in the spinal cord and pons as early as 3 months, but more consistent and numerous by 6 months. Tangles morphologically similar to those in JNPL3 mice but older bigenic female mice had a marked increase in neurofibrillary tangles in limbic areas by 6 months, especially the olfactory cortex, entorhinal cortex and amygdala (Lewis et al., 2001).

Gliosis

Reactive astrocytes and microglia as early as 3 months in the hippocampus as measured by GFAP and CD45. Increased astrocytosis with age especially in limbic areas with the most neurofibrillary tangles. Microglia especially concentrated around plaques at 9 and 12 months (Lewis et al., 2001).

Synaptic Loss

Unknown.

Last Updated: 25 Nov 2019

COMMENTS / QUESTIONS

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Further Reading

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Research Models

PDAPP(line109)

Synonyms: hAPP695Indiana, elan mouse, PDAPP, PD-APP

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Species: Mouse
Genes: APP
Modification: APP: Transgenic
Disease Relevance: Alzheimer's Disease
Strain Name: N/A

Summary

Phenotype Characterization

When visualized, these models will distributed over a 18 month timeline demarcated at the following intervals: 1mo, 3mo, 6mo, 9mo, 12mo, 15mo, 18mo+.

Absent

  • Tangles
  • Neuronal Loss

No Data

Plaques

In heterozygous mice no plaque pathology at 4-6 months. At 6-9 months mice begin to exhibit deposits of human Aβ in the hippocampus, corpus callosum, and cerebral cortex. Plaques become more extensive with age and vary in size and structure including diffuse irregular plaques and compact cored plaques (Games et al., 1995).

Tangles

No paired helical filaments or aggregates, but phosphorylated tau immunoreactivity is observed in dystrophic neurites after 14 months (Masliah et al., 2001).

Neuronal Loss

Absent.

Gliosis

GFAP-positive astrocytes and activated microglia associated with plaques (Games et al., 1995).

Synaptic Loss

Decreased synaptic density in the dentate gyrus as measured by synaptophysin immunoreactivity. Also decreased dendritic density as measured by MAP2 immunoreactivity (Games et al., 1995).

Changes in LTP/LTD

Alterations in LTP induced by theta burst stimulation at 4-5 months which is prior to plaque formation; although the potentiation immediately after TBS was comparable to control mice, the potentiation decayed more rapidly in PDAPP mice. Also paired pulse facilitation was enhanced. Responses to high frequency stimulation bursts were distorted (Larson et al., 1999).

Cognitive Impairment

Deficits in a variety of memory paradigms from a young age. Robust deficits in the radial arm maze at 3 months (deficits appear before amyloid plaque deposits). Object recognition, 6, 9-10 months. Operant learning, 3, 6 months (Dodart et al., 1999).

Last Updated: 31 Mar 2022

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Further Reading

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Research Models

APPSwDI x NOS2 Knock-out

Synonyms: APPSwDI/NOS2 bigenic mice, APPSDI/NOS2KO, CVN

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Species: Mouse
Genes: APP, NOS2
Modification: APP: Transgenic; NOS2: Knock-Out
Disease Relevance: Alzheimer's Disease
Strain Name: B6.Cg-Nos2tm1Lau Tg(Thy1-APPSwDutIowa)BWevn/Mmjax

Neuropathology

Abundant plaques, especially in the thalamus and subiculum. Aggregated, hyperphosphorylated tau tangles. Neuronal loss, especially of neuropeptide Y (NPY) neurons in the hippocampus and subiculum. Compared to Tg-SwDI mice, the bigenic mice exhibit more severe pathology.

Cognition/Behavior

Severe learning and memory deficits. Impaired spatial memory compared to APPSwDI as measured by the radial arm maze and the Barnes maze at 52-56 weeks.

Phenotype Characterization

When visualized, these models will distributed over a 18 month timeline demarcated at the following intervals: 1mo, 3mo, 6mo, 9mo, 12mo, 15mo, 18mo+.

Absent

No Data

  • Gliosis
  • Synaptic Loss
  • Changes in LTP/LTD

Plaques

Aβ deposits by 52 weeks. Particularly dense Aβ immunoreactivity in the subiculum and thalamus, including in the cerebral microvessels (Wilcock et al., 2008).

Tangles

Extensive tau pathology by 52 weeks, including intraneuronal aggregates of hyperphosphorylated tau. Increased phosphorylated tau in bigenic mice compared to APPSwDI mice (Wilcock et al., 2008).

Neuronal Loss

Significant neuron loss by 52 weeks in the hippocampus and subiculum, especially of neuropeptide Y neurons. Numerous Fluoro-Jade C+ neurons: 30% loss in the hippocampus, 35% loss in the subiculum (Wilcock et al., 2008).

Gliosis

Unknown.

Synaptic Loss

Unknown.

Changes in LTP/LTD

Unknown.

Cognitive Impairment

Impairments in spatial memory by 52-56 weeks as measured by the radial arm maze and the Barnes maze. Bigenic mice more impaired than APPSwDI (Wilcock et al., 2008).

Last Updated: 06 Apr 2022

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Therapeutics

Prednisone

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Overview

Name: Prednisone
Therapy Type: Small Molecule (timeline)
Target Type: Inflammation (timeline)
Condition(s): Alzheimer's Disease
U.S. FDA Status: Alzheimer's Disease (Discontinued)

Background

Prednisone is a corticosteroid drug that is being prescribed in many countries for conditions ranging from severe allergic reactions to arthritis, asthma, and certain autoimmune disorders such as multiple sclerosis and lupus. Prednisone temporarily attracted interest in Alzheimer's research in the late 1990s as a potential treatment option to test the inflammatory hypothesis of Alzheimer's disease. This approach failed and therapeutic testing moved on to non-steroidal anti-inflammatory drugs such as naproxen and ibuprofen.

Findings

The Alzheimer's Disease Cooperative Study conducted a multicenter trial of 138 patients with Alzheimer's disease who were randomized to take placebo or 20 mg predisone per day for a month, followed by 10 mg daily for a year and gradual tapering off over an additional four months. Treated patients showed no benefit over placebo recipients on the primary outcome, the ADAS-cog, or on a secondary analysis of only those patients who completed the full course. The main differences between the placebo and treatment groups were a worsening of behavioral symptoms and an increase in blood glucose levels on prednisone (Aisen et al., 2000; Koch and Szecsey, 2000; Aisen et al., 2003). 

This trial ended clinical evaluation of prednisone as an Alzheimer's therapy. Even so, a small study subsequently reported reduction of CSF AD biomarkers with prednisone treatment of other diseases, and an argument was made for short-term, high-dose intrathecal infusion of prednisone in AD (Tokuda et al., 2002; Alisky, 2008). Long-term use of prednisone has been reported to cause a reversible form of dementia (Sacks and Shulman, 2005).

Last Updated: 02 Jun 2014

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Therapeutics

AQW051

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Overview

Name: AQW051
Synonyms: Alpha-7 nicotinic acetylcholine receptor agonist
Therapy Type: Small Molecule (timeline)
Target Type: Cholinergic System (timeline)
Condition(s): Alzheimer's Disease, Parkinson's Disease, Schizophrenia
U.S. FDA Status: Alzheimer's Disease (Inactive), Parkinson's Disease (Phase 2), Schizophrenia (Phase 2)
Company: Novartis Pharmaceuticals Corporation

Background

AQW 051 is an α7 nicotinic acetylcholine receptor (nAChR) agonist for the oral treatment of cognitive deficits in schizophrenia, Alzheimer's, and Parkinson's diseases. Very little is known about this drug.

Findings

Phase 1 for this compound was conducted in Japan but no further development in this country has been reported.

Development of AQW 051 for Alzheimer's disease appears to be inactive. Clinicaltrial.gov lists one phase 2 trial in 54 patients with mild AD, which began in 2007 and was terminated in 2009.

Development of AQW 051 for cognitive symptoms in schizophrenia began in 2009 with a phase 1/2 study comparing a single dose to placebo in 68 adult patients. It continued in 2010 with a phase 2 dose-finding study comparing 2, 15 and 100 mg/d to placebo on cognitive function in 57 patients with chronic, stable schizophrenia. In September 2012, a third, 12-week phase 2 study began; it compares 10 mg/d of study drug to placebo in 134 adults with schizophrenia. This proof-of-principle study is expected to be complete in December 2013.

In addition, this compound has been tested in a phase 2 trial for its ability to reduce L-dopa induced dyskinesia in people with moderate to advanced Parkinson's disease. This 71-patient trial began in 2011 and ended in 2013.

No results of AQW 051 studies have been posted on clinicaltrials.gov or published in the peer-reviewed literature. For all AQW 051 trials, see clinicaltrials.gov

A phase II study was initiated in December 2007 to investigate the efficacy of AQW 051 treatment in patients with either mild Alzheimer's disease or amnestic mild cognitive impairment (NCT00582855). However, the trial was discontinued in June 2009. The study was being conducted in the UK and Canada 4.

Previously, phase I studies of the product were also conducted in healthy volunteers in Japan (NCT00409500) and the UK (NCT00418002) 5 6.

Drug-induced dyskinesia: Novartis completed a phase II trial of AQW 501 in patients with Parkinson's disease in February 2013 (NCT01474421). The study evaluated the safety, tolerability and efficacy of the agent in treating moderate-to-severe L-dopa induced dyskinesias. A total of 90 patients were enrolled in the US, France, Germany and Italy 7.

Last Updated: 24 Oct 2023

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Therapeutics

ABT-089

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Overview

Name: ABT-089
Therapy Type: Small Molecule (timeline)
Target Type: Cholinergic System (timeline)
Condition(s): Alzheimer's Disease
U.S. FDA Status: Alzheimer's Disease (Discontinued)
Company: Abbott Laboratories

Background

Last Updated: 10 Aug 2013

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