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Proteinopathy
In medicine, proteinopathy (; 'pref''. protein -pathy 'suff''. disease proteinopathies ''pl''.; proteinopathic ''adj''), or proteopathy, protein conformational disorder, or protein misfolding disease refers to a class of diseases in which certain proteins become structurally abnormal, and thereby disrupt the function of cells, tissues and organs of the body. Often the proteins fail to fold into their normal configuration; in this misfolded state, the proteins can become toxic in some way (a toxic gain-of-function) or they can lose their normal function. The proteinopathies include such diseases as Creutzfeldt–Jakob disease and other prion diseases, Alzheimer's disease, Parkinson's disease, amyloidosis, multiple system atrophy, and a wide range of other disorders. The term ''proteopathy'' was first proposed in 2000 by Lary Walker and Harry LeVine. The concept of proteopathy can trace its origins to the mid-19th century, when, in 1854, Rudolf Virchow coined the term amyl ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Amyloidosis
Amyloidosis is a group of diseases in which abnormal proteins, known as amyloid fibrils, build up in tissue. There are several non-specific and vague signs and symptoms associated with amyloidosis. These include fatigue, peripheral edema, weight loss, shortness of breath, palpitations, and Orthostatic hypotension, feeling faint with standing. In AL amyloidosis, specific indicators can include enlargement of the tongue and periorbital purpura. In wild-type ATTR amyloidosis, non-cardiac symptoms include: bilateral carpal tunnel syndrome, lumbar spinal stenosis, biceps tendon rupture, Small fiber peripheral neuropathy, small fiber neuropathy, and autonomic dysfunction. There are about 36 different types of amyloidosis, each due to a specific Proteopathy, protein misfolding. Within these 36 proteins, 19 are grouped into Organ-limited amyloidosis, localized forms, 14 are grouped as Systemic disease, systemic forms, and 3 proteins can identify as either. These proteins can become irre ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Prion
Prions are misfolded proteins that have the ability to transmit their misfolded shape onto normal variants of the same protein. They characterize several fatal and transmissible neurodegenerative diseases in humans and many other animals. It is not known what causes a normal protein to misfold, but the resulting abnormal three-dimensional structure confers infectious properties by collapsing nearby protein molecules into the same shape. The word ''prion'' is derived from the term, "proteinaceous infectious particle". In comparison to all other known infectious agents such as viroids, viruses, bacteria, fungi, and parasites, all of which contain nucleic acids ( DNA, RNA, or both), the hypothesized role of a protein as an infectious agent stands in contrast. Prion isoforms of the prion protein (PrP), whose specific function is uncertain, are hypothesized as the cause of transmissible spongiform encephalopathies (TSEs), including scrapie in sheep, chronic wasting disease (CWD) ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Parkinson's Disease
Parkinson's disease (PD), or simply Parkinson's, is a long-term degenerative disorder of the central nervous system that mainly affects the motor system. The symptoms usually emerge slowly, and as the disease worsens, non-motor symptoms become more common. The most obvious early symptoms are tremor, rigidity, slowness of movement, and difficulty with walking. Cognitive and behavioral problems may also occur with depression, anxiety, and apathy occurring in many people with PD. Parkinson's disease dementia becomes common in the advanced stages of the disease. Those with Parkinson's can also have problems with their sleep and sensory systems. The motor symptoms of the disease result from the death of cells in the substantia nigra, a region of the midbrain, leading to a dopamine deficit. The cause of this cell death is poorly understood, but involves the build-up of misfolded proteins into Lewy bodies in the neurons. Collectively, the main motor symptoms are also known as ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Fibrillar
Fibrils (from the Latin ''fibra'') are structural biological materials found in nearly all living organisms. Not to be confused with fibers or filaments, fibrils tend to have diameters ranging from 10-100 nanometers (whereas fibers are micro to milli-scale structures and filaments have diameters approximately 10-50 nanometers in size). Fibrils are not usually found alone but rather are parts of greater hierarchical structures commonly found in biological systems. Due to the prevalence of fibrils in biological systems, their study is of great importance in the fields of microbiology, biomechanics, and materials science. Structure and mechanics Fibrils are composed of linear biopolymers, and are characterized by rod-like structures with high length-to-diameter ratios. They often spontaneously arrange into helical structures. In biomechanics problems, fibrils can be characterized as classical beams with a roughly circular cross-sectional area on the nanometer scale. As such, ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Congo Red
Congo red is an organic compound, the sodium salt of 3,3′-( ,1′-biphenyl4,4′-diyl)bis(4-aminonaphthalene-1-sulfonic acid). It is an azo dye. Congo red is water-soluble, yielding a red colloidal solution; its solubility is greater in organic solvents. However, the use of Congo red has long been abandoned, primarily because of its carcinogenic properties.Klaus Hunger, Peter Mischke, Wolfgang Rieper, Roderich Raue, Klaus Kunde, Aloys Engel: "Azo Dyes" in ''Ullmann’s Encyclopedia of Industrial Chemistry'', 2005, Wiley-VCH, Weinheim.. History Congo red was first synthesized in 1883 by Paul Böttiger, who had been employed at Friedrich Bayer Company in Elberfeld, Germany. He was looking for textile dyes that did not require a mordant step. The company which had a right of first refusal to his inventions was not interested in this bright red color, so he filed the patent under his own name and sold it to the AGFA company of Berlin. AGFA marketed the dye under the name "Congo ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Polarized Light
Polarization (also polarisation) is a property applying to transverse waves that specifies the geometrical orientation of the oscillations. In a transverse wave, the direction of the oscillation is perpendicular to the direction of motion of the wave. A simple example of a polarized transverse wave is vibrations traveling along a taut string ''(see image)''; for example, in a musical instrument like a guitar string. Depending on how the string is plucked, the vibrations can be in a vertical direction, horizontal direction, or at any angle perpendicular to the string. In contrast, in longitudinal waves, such as sound waves in a liquid or gas, the displacement of the particles in the oscillation is always in the direction of propagation, so these waves do not exhibit polarization. Transverse waves that exhibit polarization include electromagnetic waves such as light and radio waves, gravitational waves, and transverse sound waves (shear waves) in solids. An electromagnetic wave ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Birefringence
Birefringence is the optical property of a material having a refractive index that depends on the polarization and propagation direction of light. These optically anisotropic materials are said to be birefringent (or birefractive). The birefringence is often quantified as the maximum difference between refractive indices exhibited by the material. Crystals with non-cubic crystal structures are often birefringent, as are plastics under mechanical stress. Birefringence is responsible for the phenomenon of double refraction whereby a ray of light, when incident upon a birefringent material, is split by polarization into two rays taking slightly different paths. This effect was first described by Danish scientist Rasmus Bartholin in 1669, who observed it in calcite, a crystal having one of the strongest birefringences. In the 19th century Augustin-Jean Fresnel described the phenomenon in terms of polarization, understanding light as a wave with field components in transverse polariz ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Friedrich August Kekulé Von Stradonitz
Friedrich may refer to: Names * Friedrich (surname), people with the surname ''Friedrich'' * Friedrich (given name), people with the given name ''Friedrich'' Other * Friedrich (board game), a board game about Frederick the Great and the Seven Years' War * ''Friedrich'' (novel), a novel about anti-semitism written by Hans Peter Richter * Friedrich Air Conditioning, a company manufacturing air conditioning and purifying products *, a German cargo ship in service 1941-45 See also * Friedrichs (other) * Frederick (other) * Nikolaus Friedreich {{disambig ja:フリードリヒ ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Friedreich
Nikolaus Friedreich (1 July 1825 in Würzburg – 6 July 1882 in Heidelberg) was a German pathologist and neurologist, and a third generation physician in the Friedreich family. His father was psychiatrist Johann Baptist Friedreich (1796–1862), and his grandfather was pathologist Nicolaus Anton Friedreich (1761–1836), who is remembered for his early description of idiopathic facial paralysis, which would later be known as Bell's palsy. @ Biography In the early part of his career he studied and practiced medicine at the |
Cellulose
Cellulose is an organic compound with the formula , a polysaccharide consisting of a linear chain of several hundred to many thousands of β(1→4) linked D-glucose units. Cellulose is an important structural component of the primary cell wall of green plants, many forms of algae and the oomycetes. Some species of bacteria secrete it to form biofilms. Cellulose is the most abundant organic polymer on Earth. The cellulose content of cotton fiber is 90%, that of wood is 40–50%, and that of dried hemp is approximately 57%. Cellulose is mainly used to produce paperboard and paper. Smaller quantities are converted into a wide variety of derivative products such as cellophane and rayon. Conversion of cellulose from energy crops into biofuels such as cellulosic ethanol is under development as a renewable fuel source. Cellulose for industrial use is mainly obtained from wood pulp and cotton. Some animals, particularly ruminants and termites, can digest cellulose with the help of ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Corpora Amylacea
Corpora amylacea (CA) (from the Latin meaning "starch-like bodies") is a general term for small hyaline masses found in the prostate gland, nervous system, lung, and sometimes in other organs of the body. Corpora amylacea increase in number and size with advancing age, although this increase varies from person to person. In the nervous system, they are particularly abundant in certain neurodegenerative diseases. While their significance is largely unknown, some researchers have suggested that corpora amylacea play a role in the clearance of debris. The composition and appearance of corpora amylacea can differ in different organs. In the prostate gland, where they are also known as prostatic concretions, corpora amylacea are rich in aggregated protein that has many of the features of amyloid, whereas those in the central nervous system are generally smaller and do not contain amyloid. Corpora amylacea in the central nervous system occur in the foot processes of astrocytes, and t ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Amyloid
Amyloids are aggregates of proteins characterised by a Fibril, fibrillar morphology of 7–13 Nanometer, nm in diameter, a beta sheet (β-sheet) Secondary structure of proteins, secondary structure (known as cross-β) and ability to be Staining, stained by particular dyes, such as Congo red. In the human body, amyloids have been linked to the development of various diseases. Pathogenic amyloids form when previously healthy proteins lose their normal Protein structure, structure and physiology, physiological functions (Protein misfolding, misfolding) and form fibrous deposits in amyloid plaques around cells which can disrupt the healthy function of tissues and organs. Such amyloids have been associated with (but not necessarily as the cause of) more than 50 human diseases, known as amyloidosis, and may play a role in some neurodegenerative diseases. Some of these diseases are mainly sporadic and only a few cases are Genetic disorder, familial. Others are only Genetic disorder, fam ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
