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Lytico-bodig Disease
Lytico-bodig (also Lytigo-bodig) disease, Guam disease, or amyotrophic lateral sclerosis-parkinsonism-dementia (ALS-PDC) is a neurodegenerative disease of uncertain etiology endemic to the Chamorro people of the island of Guam in Micronesia. ''Lytigo'' and ''bodig'' are Chamorro language words for two different manifestations of the same condition. ''ALS-PDC'', a term coined by Asao Hirano and colleagues in 1961, reflects its resemblance to amyotrophic lateral sclerosis (ALS), Parkinson's disease, and Alzheimer's disease. First reports of the disease surfaced in three death certificates on Guam in 1904 which made some mention of paralysis. The frequency of cases grew amongst the Chamorro until it was the leading cause of adult death between 1945 and 1956. The incidence rate was 200 per 100,000 per year and it was 100 times more prevalent than in the rest of the world. Neurologist Oliver Sacks detailed this disease in his book ''The Island of the Colorblind''. Sacks and Paul Alan Cox ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Neurodegeneration
A neurodegenerative disease is caused by the progressive loss of structure or function of neurons, in the process known as neurodegeneration. Such neuronal damage may ultimately involve cell death. Neurodegenerative diseases include amyotrophic lateral sclerosis, multiple sclerosis, Parkinson's disease, Alzheimer's disease, Huntington's disease, multiple system atrophy, and prion diseases. Neurodegeneration can be found in the brain at many different levels of neuronal circuitry, ranging from molecular to systemic. Because there is no known way to reverse the progressive degeneration of neurons, these diseases are considered to be incurable; however research has shown that the two major contributing factors to neurodegeneration are oxidative stress and inflammation. Biomedical research has revealed many similarities between these diseases at the subcellular level, including atypical protein assemblies (like proteinopathy) and induced cell death. These similarities suggest that ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Frontotemporal Degeneration
Frontotemporal dementia (FTD), or frontotemporal degeneration disease, or frontotemporal neurocognitive disorder, encompasses several types of dementia involving the progressive degeneration of frontal and temporal lobes. FTDs broadly present as behavioral or language disorders with gradual onsets. The three main subtypes or variant syndromes are a behavioral variant (bvFTD) previously known as ''Pick's disease'', and two variants of primary progressive aphasia – semantic variant (svPPA), and nonfluent variant (nfvPPA). Two rare distinct subtypes of FTD are neuronal intermediate filament inclusion disease (NIFID), and basophilic inclusion body disease. Other related disorders include corticobasal syndrome and FTD with amyotrophic lateral sclerosis (ALS) ''FTD-ALS'' also called ''FTD- MND''. Frontotemporal dementias are mostly early-onset syndromes that are linked to frontotemporal lobar degeneration (FTLD), which is characterized by progressive neuronal loss predominantly i ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Kuru (disease)
Kuru is a rare, incurable, and fatal Neurological disorder, neurodegenerative disorder that was formerly common among the Fore people of Papua New Guinea. Kuru is a form of transmissible spongiform encephalopathy (TSE) caused by the transmission of abnormally folded proteins (prions), which leads to symptoms such as tremors and loss of coordination from neurodegeneration. The term kuru derives from the Fore language, Fore word ''kuria'' or ''guria'' ("to shake"), due to the body tremors that are a classic symptom of the disease. ''Kúru'' itself means "trembling". It is also known as the "laughing sickness" due to the pathologic bursts of laughter which are a symptom of the disease. It is now widely accepted that kuru was transmitted among members of the Fore tribe of Papua New Guinea via Endocannibalism, funerary cannibalism. Deceased family members were traditionally cooked and eaten, which was thought to help free the spirit of the dead. Women and children usually consumed the ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
L-DOPA
-DOPA, also known as levodopa and -3,4-dihydroxyphenylalanine, is an amino acid that is made and used as part of the normal biology of some plants and animals, including humans. Humans, as well as a portion of the other animals that utilize -DOPA, make it via biosynthesis from the amino acid -tyrosine. -DOPA is the precursor to the neurotransmitters dopamine, norepinephrine (noradrenaline), and epinephrine (adrenaline), which are collectively known as catecholamines. Furthermore, -DOPA itself mediates neurotrophic factor release by the brain and CNS. -DOPA can be manufactured and in its pure form is sold as a psychoactive drug with the INN levodopa; trade names include Sinemet, Pharmacopa, Atamet, and Stalevo. As a drug, it is used in the clinical treatment of Parkinson's disease and dopamine-responsive dystonia. -DOPA has a counterpart with opposite chirality, -DOPA. As is true for many molecules, the human body produces only one of these isomers (the -DOPA form). The ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Substantia Nigra
The substantia nigra (SN) is a basal ganglia structure located in the midbrain that plays an important role in reward and movement. ''Substantia nigra'' is Latin for "black substance", reflecting the fact that parts of the substantia nigra appear darker than neighboring areas due to high levels of neuromelanin in dopaminergic neurons. Parkinson's disease is characterized by the loss of dopaminergic neurons in the substantia nigra pars compacta. Although the substantia nigra appears as a continuous band in brain sections, anatomical studies have found that it actually consists of two parts with very different connections and functions: the pars compacta (SNpc) and the pars reticulata (SNpr). The pars compacta serves mainly as a projection to the basal ganglia circuit, supplying the striatum with dopamine. The pars reticulata conveys signals from the basal ganglia to numerous other brain structures. Structure The substantia nigra, along with four other nuclei, is part ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Neurofibrillary Tangle
Neurofibrillary tangles (NFTs) are aggregates of hyperphosphorylated tau protein that are most commonly known as a primary biomarker of Alzheimer's disease. Their presence is also found in numerous other diseases known as tauopathies. Little is known about their exact relationship to the different pathologies. Formation Neurofibrillary tangles are formed by hyperphosphorylation of a microtubule-associated protein known as tau, causing it to aggregate, or group, in an insoluble form. (These aggregations of hyperphosphorylated tau protein are also referred to as PHF, or " paired helical filaments"). The precise mechanism of tangle formation is not completely understood, and it is still controversial whether tangles are a primary causative factor in disease or play a more peripheral role. Cytoskeletal changes Three different maturation states of NFT have been defined using anti-tau and anti-ubiquitin immunostaining. At stage 0 there are morphologically normal pyramidal cells showing ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Bioaccumulation
Bioaccumulation is the gradual accumulation of substances, such as pesticides or other chemicals, in an organism. Bioaccumulation occurs when an organism absorbs a substance at a rate faster than that at which the substance is lost or eliminated by catabolism and excretion. Thus, the longer the biological half-life of a toxic substance, the greater the risk of chronic poisoning, even if environmental levels of the toxin are not very high. Bioaccumulation, for example in fish, can be predicted by models. Hypothesis for molecular size cutoff criteria for use as bioaccumulation potential indicators are not supported by data. Biotransformation can strongly modify bioaccumulation of chemicals in an organism. Toxicity induced by metals is associated with bioaccumulation and biomagnification. Storage or uptake of metals faster than the rate at which an organism metabolizes and excretes lead to the accumulation of that metal. The presence of various chemicals and harmful substances in ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Guam Flying Fox
The Guam flying fox (''Pteropus tokudae''), also known as the little Marianas fruit bat, was a tiny megabat from Guam in the Marianas Islands in Micronesia that was confirmed extinct due to hunting or habitat changes. It was first recorded in 1931 and was observed roosting with the larger and much more common Mariana fruit bat. The last specimen was a female found roosting at Tarague cliff in March 1967, but it escaped capture. An unconfirmed sighting took place sometime during the 1970s and no other individuals have been sighted since then. Description The Guam flying fox has a length of about , a wingspan of about , and a body weight of . It is very similar in appearance to the Chuuk flying fox ('' Pteropus insularis''). The top of the head is greyish, the back, throat and underparts brown or dark brown and the side of the neck golden-brown. Behaviour Little is known of the behaviour of this flying fox but it is likely to have fed on the fruits, flowers and foliage of evergreen ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
BMAA
β-Methylamino--alanine, or BMAA, is a non-proteinogenic amino acid produced by cyanobacteria. BMAA is a neurotoxin and its potential role in various neurodegenerative disorders is the subject of scientific research. Structure and properties BMAA is a derivative of the amino acid alanine with a methylamino group on the side chain. This non-proteinogenic amino acid is classified as a polar base. Sources and detection BMAA is produced by cyanobacteria in marine, freshwater, and terrestrial environments. In cultured non-nitrogen-fixing cyanobacteria, BMAA production increases in a nitrogen-depleted medium. The biosynthetic pathway in cyanobacteria is unknown, but involvement of BMAA and its structural analog 2,4-diaminobutanoic acid (2,4-DAB) in envioronmental iron scavenging has been hypothesized. BMAA has been found in aquatic organisms and in plants with cyanobacterial symbionts such as certain lichens, the floating fern ''Azolla'', the leaf petioles of the tropical fl ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
ODAP
Oxalyldiaminopropionic acid (ODAP) is a structural analogue of the neurotransmitter glutamate found in the grass pea ''Lathyrus sativus''. It is the neurotoxin responsible for the motor neuron degeneration syndrome lathyrism. Sources ODAP is found in the seeds of the legume ''L. sativus'', a grass pea plant, in the range of .5% w/w. ''L. sativus'' can be found in areas of Southern, Central, and Eastern Europe, the Mediterranean Basin, Iraq and Afghanistan as well as areas of Asia and Africa.Heuzé V., Tran G., Hassoun P., Lessire M., Lebas F., 2016. Grass pea (Lathyrus sativus). Feedipedia, a programme by INRA, CIRAD, AFZ and FAO. https://www.feedipedia.org/node/285 Last updated on April 19, 2016, 15:36 History In some regions, including the Indian subcontinent, Bangladesh, Ethiopia and Nepal, the grass pea has become a staple food item. The plant has a high tolerance of environmental conditions which results in it being the only available food source in times of famine or drought ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lathyrism
Lathyrism is a condition caused by eating certain legumes of the genus ''Lathyrus''. There are three types of lathyrism: ''neurolathyrism'', ''osteolathyrism'', and ''angiolathyrism'', all of which are incurable, differing in their symptoms and in the body tissues affected. Neurolathyrism is the type associated with the consumption of legumes in the genus ''Lathyrus'' that contain the toxin ODAP. ODAP ingestion results in motorneuron death. The result is paralysis and muscle atrophy of the lower limbs. Osteolathyrism, a different type of lathyrism, affects the connective tissues, not the motorneurons. Osteolathyrism results from the ingestion of ''Lathyrus odoratus'' seeds (sweet peas), and is often referred to as odoratism. It is caused by a different toxin, beta-aminopropionitrile, which affects the linking of the subunits of collagen, a major structural protein found in connective tissue. A third type of lathyrism is angiolathyrism, which is similar to osteolathyrism in its ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Cycasin
Cycasin is a carcinogenic and neurotoxic glucoside found in cycads such as ''Cycas revoluta'' and ''Zamia pumila''. Symptoms of poisoning include vomiting, diarrhea, weakness, seizures, and hepatotoxicity. In metabolic conditions, cycasin is hydrolyzed into glucose and methylazoxymethanol (MAM), the latter of which dissociates into formaldehyde and diazomethane. It induces hepatotoxicity and Zamia staggers, a fatal nervous disease affecting cattle resulting from browsing on the leaves or other parts of cycads. Sources Cycasin is found in all known cycad genera and is distributed throughout the body of the plant, but with the highest concentration in the seeds. It is one of several toxins found in cycad plants, along with the neurotoxic amino acid BMAA. The origin and biological role of these toxins is unknown, as there does not appear to be a statistically significant correlation between the concentration of toxic material and the types of herbivory observed in animals consuming th ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
