Protein Degradation in Health and Disease (Progress in Molecular and Subcellular Biology)
Lee B. Molecular Genetics of Dysregulated pH Homeostasis. Jen-Tsan Ashley Chi.
Armen Parsyan. Ubiquitination and Transmembrane Signaling. Sudha K. Hormone Resistance and Other Endocrine Paradoxes. Margo P. Hard Tissue Mineralization and Demineralization. Norimitsu Watabe.
International Review of Cell and Molecular Biology. Kwang W. Novel Approaches to Treatment of Osteoporosis. Daniel Klionsky. Cytokine Frontiers. Takayuki Yoshimoto.
Experimental Hematology Today. Therapeutic Angiogenesis. Yukihito Higashi. Lorenzo Galluzzi. Dhyan Chandra. Epigenetic Mechanisms in Cellular Reprogramming. Alexander Meissner. Basics of Human Andrology.
Journal of Biochemistry and Molecular Biology Research
Anand Kumar. Prehistoric Human Bone. Gisela Grupe. Studies on Pediatric Disorders. Hirokazu Tsukahara. The Cerebral Perivascular Cells. Doychin N.
SUMOylation and Ubiquitination: Current and Emerging Concepts
Rudolf Toman. Cell Immortalization. Alvaro Macieira-Coelho. Extracellular Matrix in Tumor Biology. Rolf A. Gordon S. Danny R Welch. Strategies in Regenerative Medicine. Matteo Santin. Molecular Biology of the Male Reproductive System. David de Kretser. Carl Wu. Walter Doerfler. Gaetano Santulli. Regenerative Medicine and Cell Therapy. Hossein Baharvand. Cell-Extracellular Matrix Interactions in Cancer. Roy Zent. Joseph G. Mariusz Z. Biomaterials for Musculoskeletal Regeneration.
Bikramjit Basu. Lynne E. This loss results in gross atrophy of the affected regions, including degeneration in the temporal lobe and parietal lobe , and parts of the frontal cortex and cingulate gyrus. Alzheimer's disease has been hypothesized to be a protein misfolding disease proteopathy , caused by accumulation of abnormally folded A-beta and tau proteins in the brain.
Beta-amyloid is a fragment from a larger protein called amyloid precursor protein APP , a transmembrane protein that penetrates through the neuron's membrane. APP is critical to neuron growth, survival and post-injury repair. Parkinson's disease is the second most common neurodegenerative disorder  and manifests as bradykinesia, rigidity, resting tremor and posture instability.
The crude prevalence rate of PD has been reported to range from 15 per , to 12, per ,, and the incidence of PD from 15 per , to per ,, with the disease being less common in Asian countries. Parkinson's disease is a degenerative disorder of the central nervous system. It results from the death of dopamine-generating cells in the substantia nigra, a region of the midbrain; the cause of cell-death is unknown.
The following paragraph is an excerpt from the Pathophysiology section of the article Parkinson's disease.
The mechanism by which the brain cells in Parkinson's are lost may consist of an abnormal accumulation of the protein alpha-synuclein bound to ubiquitin in the damaged cells. The alpha-synuclein -ubiquitin complex cannot be directed to the proteasome.
This protein accumulation forms proteinaceous cytoplasmic inclusions called Lewy bodies. The latest research on pathogenesis of disease has shown that the death of dopaminergic neurons by alpha-synuclein is due to a defect in the machinery that transports proteins between two major cellular organelles — the endoplasmic reticulum ER and the Golgi apparatus. Certain proteins like Rab1 may reverse this defect caused by alpha-synuclein in animal models.
Recent research suggests that impaired axonal transport of alpha-synuclein leads to its accumulation in the Lewy bodies.
- PROTEIN DEGRADATION IN HEALTH AND DISEASE: REBOUD-RAVAUX M.?
- The Political Economy of Human Rights Enforcement: Moral and Intellectual Leadership in the Context of Global Hegemony.
- New insights into the cellular temporal response to proteostatic stress | eLife;
- The Power of Business Process Improvement: 10 Simple Steps to Increase Effectiveness, Efficiency, and Adaptability;
Experiments have revealed reduced transport rates of both wild-type and two familial Parkinson's disease-associated mutant alpha-synucleins through axons of cultured neurons. The main known risk factor is age. The following paragraph is an excerpt from the Mechanism section of the article Huntington's disease. HD causes astrogliosis  and loss of medium spiny neurons. The areas affected are mainly in the striatum , but also the frontal and temporal cortices.
The weaker signals from subthalamic nuclei thus cause reduced initiation and modulation of movement, resulting in the characteristic movements of the disorder, notably chorea. Mutant Huntingtin is an aggregate-prone protein. During the cells' natural clearance process, these proteins are retrogradely transported to the cell body for destruction by lysosomes.
It is a possibility that these mutant protein aggregates damage the retrograde transport of important cargoes such as BDNF by damaging molecular motors as well as microtubules. Amyotrophic lateral sclerosis ALS or Lou Gehrig's disease is a disease in which motor neurons are selectively targeted for degeneration. This discovery led researchers to focus on unlocking the mechanisms for SOD1-mediated diseases. However, the pathogenic mechanism underlying SOD1 mutant toxicity has yet to be resolved.
More recently, TDP and FUS protein aggregates have been implicated in some cases of the disease, and a mutation in chromosome 9 C9orf72 is thought to be the most common known cause of sporadic ALS.
- Browse more videos.
- Protein Degradation in Health and Disease.
- Top Authors.
- Bibliographic Information.
- Protein folding in the cell | SALAHUDDIN | Journal of Biochemistry and Molecular Biology Research.
- SUMOylation and Ubiquitination: Current and Emerging Concepts | Book;
Recent independent research by Nagai et al. Astrocytes then cause the toxic effects on the motor neurons. The specific mechanism of toxicity still needs to be investigated, but the findings are significant because they implicate cells other than neuron cells in neurodegeneration.