The Role of the Amyloid Precursor Protein (APP) Gene Family for the Development and Function of the Neuromuscular Junction

The Role of the Amyloid Precursor Protein (APP) Gene Family for the Development and Function of the Neuromuscular Junction PDF Author: Maja Klevanski
Publisher:
ISBN:
Category :
Languages : en
Pages : 151

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The Role of the Amyloid Precursor Protein (APP) Gene Family for the Development and Function of the Neuromuscular Junction

The Role of the Amyloid Precursor Protein (APP) Gene Family for the Development and Function of the Neuromuscular Junction PDF Author: Maja Klevanski
Publisher:
ISBN:
Category :
Languages : en
Pages : 151

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The Physiological Functions of the Amyloid Precursor Protein Gene Family

The Physiological Functions of the Amyloid Precursor Protein Gene Family PDF Author: Ulrike C. Müller
Publisher: Frontiers Media SA
ISBN: 2889453553
Category :
Languages : en
Pages : 295

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Book Description
The amyloid precursor protein APP plays a key role in the pathogenesis of Alzheimer’s disease (AD), as proteolytical cleavage of APP gives rise to the Aβ peptide which is deposited in the brains of Alzheimer patients. Despite this, our knowledge of the normal cell biological and physiological functions of APP and the closely related APLPs is limited. This may have hampered our understanding of AD, since evidence has accumulated that not only the production of the Aβ peptide but also the loss of APP-mediated functions may contribute to AD pathogenesis. Thus, it appears timely and highly relevant to elucidate the functions of the APP gene family from the molecular level to their role in the intact organism, i.e. in the context of nervous system development, synapse formation and adult synapse function, as well as neural homeostasis and aging. Why is our understanding of the APP functions so limited? APP and the APLPs are multifunctional proteins that undergo complex proteolytical processing. They give rise to an almost bewildering array of different fragments that may each subserve specific functions. While Aβ is aggregation prone and neurotoxic, the large secreted ectodomain APPsα - produced in the non-amyloidogenic α-secretase pathway - has been shown to be neurotrophic, neuroprotective and relevant for synaptic plasticity, learning and memory. Recently, novel APP cleavage pathways and enzymes have been discovered that have gained much attention not only with respect to AD but also regarding their role in normal brain physiology. In addition to the various cleavage products, there is also solid evidence that APP family proteins mediate important functions as transmembrane cell surface molecules, most notably in synaptic adhesion and cell surface signaling. Elucidating in more detail the molecular mechanisms underlying these divers functions thus calls for an interdisciplinary approach ranging from the structural level to the analysis in model organisms. Thus, in this research topic of Frontiers we compile reviews and original studies, covering our current knowledge of the physiological functions of this intriguing and medically important protein family.

Amyloid Protein Precursor in Development, Aging and Alzheimer’s Disease

Amyloid Protein Precursor in Development, Aging and Alzheimer’s Disease PDF Author: C.L. Masters
Publisher: Springer Science & Business Media
ISBN: 3662011352
Category : Science
Languages : en
Pages : 277

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Book Description
This book summarizes the last ten years' research on Alzheimer's disease. Genetic mutations in the gene which codes for amyloid precursor protein (APP) have now been shown to cause Alzheimer's disease in some families. Other genetic loci are now being discovered which relate to Alzheimer's disease in some families. Understanding the normal structure and function of the APP gene product will eventually provide avenues for developing specific therapeutic strategies targeted at the amyloid deposition in the Alzheimer's disease brain. Drugs which can inhibit or dissolve the amyloid, affect the synthesis and proteolysis of APP, or which regulate the activity of the APP gene all hold the promise of eventually yielding an effective treatment for Alzheimer's disease.

Investigating the Contribution of Amyloid Precursor Protein (APP) to Pathology in a Mouse Model of Amyotrophic Lateral Sclerosis (ALS)

Investigating the Contribution of Amyloid Precursor Protein (APP) to Pathology in a Mouse Model of Amyotrophic Lateral Sclerosis (ALS) PDF Author: J. Barney N. B. Bryson
Publisher:
ISBN:
Category : Amyloid beta-protein precursor
Languages : en
Pages : 426

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In Amyotrophic Lateral Sclerosis (ALS), neuromuscular junction (NMJ) dysfunction and degeneration of spinal motor neurons causes progressive loss of skeletal muscle function, culminating in paralysis and ultimately death. Upregulation of amyloid beta (A4) precursor protein (APP) in muscle fibres coincides with symptom onset in both sporadic ALS (sALS) patients and the SOD1G93A mouse model of familial ALS. -- The results of this Thesis provide further characterization of this reactive APP response in muscle tissue from SOD1G93A mice, which revealed a clear correlation between the reactive APP-response and hallmarks of disease progression. Moreover, the results presented in this Thesis demonstrate that genetic ablation of APP in SOD1G93A mice significantly improved multiple disease parameters, including: NMJ innervation; motor function; muscle contractile characteristics; motor unit and motor neuron survival. Therefore these results strongly suggest that endogenous APP actively contributes to ALS-like pathology in SOD1G93A mice. Additionally, the results presented here demonstrate that [beta]-Amyloid (AP) peptides occur at elevated levels in spinal cord tissue and form aggregates within motor neurons in (120d) SOD1G93A mice. -- Along with previous observations of the reactive APP response in muscle tissue and accumulation of Ap peptides in motor neurons in cases of sALS, the results presented in this Thesis therefore suggest that APP upregulation and/or its amyloidogenic processing could contribute to ALS pathology irrespective of the initial trigger. Thus, targetting [sic] APP production or processing may provide a novel therapeutic strategy for ALS.

The Role of the Amyloid Precursor Protein (APP) in Protein Homeostasis and Neuroprotection

The Role of the Amyloid Precursor Protein (APP) in Protein Homeostasis and Neuroprotection PDF Author: Arpita Kundu
Publisher:
ISBN:
Category :
Languages : en
Pages :

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Book Description
Alzheimer's disease is a chronic neurodegenerative disease that causes problems with memory, thinking and behavior. The pathophysiological hallmarks of AD are extracellular senile plaques and intracellular neurofibrillary tangles. Amyloid plaques mainly contain the amyloid-[beta] (A[beta]) peptide, which appears as a cleavage product of the APP. APP is a type I transmembrane protein with a large extracellular domain and a short cytoplasmic tail. It is expressed in variety of tissues e.g. in neuronal tissue (brain, spinal cord, retina), and non-neuronal tissues (kidney, lung, pancreas, prostate gland, and thyroid gland) (Dawkins and Small, 2014). APP has been studied because of its link to AD, however, its role in normal brain function is poorly understood. APP is processed by two different pathways, amyloidogenic pathway and non-amyloidogenic pathway. ...

Assessing the Functional Role of Amyloid Precursor Protein Family Members and Primary Cilia in the Central Nervous System

Assessing the Functional Role of Amyloid Precursor Protein Family Members and Primary Cilia in the Central Nervous System PDF Author: Kang Han
Publisher:
ISBN:
Category :
Languages : en
Pages :

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The Fibroblast Growth Factor Family

The Fibroblast Growth Factor Family PDF Author: Andrew Baird
Publisher:
ISBN:
Category : Medical
Languages : en
Pages : 542

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Data Analysis in Molecular Biology and Evolution

Data Analysis in Molecular Biology and Evolution PDF Author: Xuhua Xia
Publisher: Springer Science & Business Media
ISBN: 030646893X
Category : Science
Languages : en
Pages : 284

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Book Description
Data Analysis in Molecular Biology and Evolution introduces biologists to DAMBE, a proprietary, user-friendly computer program for molecular data analysis. The unique combination of this book and software will allow biologists not only to understand the rationale behind a variety of computational tools in molecular biology and evolution, but also to gain instant access to these tools for use in their laboratories. Data Analysis in Molecular Biology and Evolution serves as an excellent resource for advanced level undergraduates or graduates as well as for professionals working in the field.

Studies of the Cell Biological Function of the Amyloid Precursor Protein (APP) Family in Drosophila Melanogaster and Mammals

Studies of the Cell Biological Function of the Amyloid Precursor Protein (APP) Family in Drosophila Melanogaster and Mammals PDF Author: Peter Šoba
Publisher:
ISBN:
Category :
Languages : en
Pages : 0

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Alzheimer's Disease

Alzheimer's Disease PDF Author: Jolanta Dorszewska
Publisher: BoD – Books on Demand
ISBN: 178923462X
Category : Medical
Languages : en
Pages : 164

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Book Description
Alzheimer's disease was discovered over 100 years ago and still belongs to incurable neurological diseases; its pharmacotherapy is considered to be ineffective. This book presents contemporary views on the genetic, biochemical, and immunological determinants of this disease. This book also concerns the issue of Alzheimer's disease prevention through lifestyle and physical activity. Moreover, it describes the therapies used in Alzheimer's disease to slow the progression of the disease and delay its onset. Subsequently, the authors discuss experimental and clinical trials used now and in the near future. We hope that this book will help the readers to understand the complex mechanism leading to the development of Alzheimer's disease and indicate effective ways to prevent this disorder.