Related Experiment Video
Updated: Apr 30, 2026
07:58
Establishment of a Surgically-induced Model in Mice to Investigate the Protective Role of Progranulin in Osteoarthritis
Published on: February 25, 2014
28.2K
Progranulin in neurodegenerative disease.
Terri L Petkau1, Blair R Leavitt2
1Centre for Molecular Medicine and Therapeutics, Department of Medical Genetics, University of British Columbia, and Children's and Women's Hospital, 980 West 28th Avenue, Vancouver, BC, Canada V5Z 4H4.
Trends in Neurosciences
|May 8, 2014
Summary
Progranulin gene mutations cause familial frontotemporal dementia (FTD). This review explores progranulin
Area of Science:
- Neuroscience
- Genetics
- Neurology
Background:
- Loss-of-function mutations in the progranulin gene are a significant cause of familial frontotemporal dementia (FTD).
- Progranulin (PGRN) plays a critical role in cellular homeostasis and lysosomal function.
- Understanding PGRN's function is crucial for developing targeted therapies for neurodegenerative diseases.
Purpose of the Study:
- To review the multifaceted role of progranulin in both healthy and diseased states.
- To examine the clinical and neuropathological characteristics of FTD patients with progranulin gene mutations.
- To synthesize evidence on progranulin's function in the brain, particularly from mouse models, to inform therapeutic strategies.
Main Methods:
- Review of clinical and neuropathological data from FTD patients with progranulin gene mutations.
- Examination of evidence for progranulin's role in brain function, with a focus on findings from mouse models.
- Synthesis of current knowledge on progranulin biology and its implications for neurodegeneration.
Main Results:
- FTD associated with progranulin mutations presents a complex phenotype, suggesting multifactorial neurodegeneration.
- Evidence indicates that genetic and environmental factors interact to influence progranulin-mediated neurodegeneration.
- Mouse models provide valuable insights into progranulin's diverse functions within the brain.
Conclusions:
- Progranulin's role in neurodegeneration is complex and influenced by various factors.
- Further understanding of progranulin biology is essential for developing effective therapies.
- Developing therapeutics that modulate endogenous progranulin levels holds promise for treating FTD and other neurodegenerative diseases.
Related Concept Videos
01:23Alzheimer Disease ll: Pathophysiology
35
Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and...
35
01:37Neural Regulation
34.8K
Digestion begins with a cephalic phase that prepares the digestive system to receive food. When our brain processes visual or olfactory information about food, it triggers impulses in the cranial nerves innervating the salivary glands and stomach to prepare for food.
34.8K
03:03Amyloid Fibrils
10.2K
Amyloid fibrils are aggregates of misfolded proteins. Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils.
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
10.2K
01:15Neurogenesis and Regeneration of Nervous Tissue
2.1K
In the CNS, neurogenesis, the birth of new neurons from stem cells, is limited to the hippocampus in adults. In other regions of the brain and spinal cord, neurogenesis is almost non-existent due to inhibitory influences from neuroglia, especially oligodendrocytes, and the absence of growth-stimulating cues. The myelin produced by oligodendrocytes in the CNS inhibits neuronal regeneration. Furthermore, astrocytes proliferate rapidly after neuronal damage, forming scar tissue that physically...
2.1K
01:24Parkinson Disease ll: Pathophysiology
28
Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...
28