| ¿µ¹® | prion | ÇÑ±Û | ÇÁ¸®¿Â |
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| ¼³¸í | ÇÁ¸®¿ÂÀº Àü¿°¼º ÇØ¸é³úº´ÁõÀ̶ó°í ºÎ¸£±âµµ Çϴµ¥ »ç¶÷¿¡¼ ¹ßº´ÇÏ´Â Å©·ÎÀÌÃ÷ÆçÆ®-¾ßÄߺ´, Gerstmann-Straussler-Scheinkerº´, Äí·ç, Ä¡¸íÀû °¡Á·ºÒ¸éÁõ°ú ¾ç°ú ¿°¼ÒÀÇ ¸é¾ç¶³¸²º´(scrapie), ¹ÖÅ©ÀÇ Àü¿°¼º ³úº´Áõ°ú ¼ÒÀÇ ÇØ¸é³úº´Áõ(±¤¿ìº´) µîÀ» Æ÷ÇÔÇÑ´Ù. ÀÌ º´µéÀº ´ëºÎºÐ ³úÀÇ È¸»öÁúÀÇ ÇØ¸é¸ð¾ç º´Å͸¦ ÀÏÀ¸Å°¸ç, ÀÓ»ó¼Ò°ßÀº »¡¸® ÁøÇàÇÏ´Â Ä¡¸ÅÀÌ´Ù. ÀÌ º´À» ¾Î°í ÀÖ´Â ¼÷ÁÖÀÇ ³úÁ¶Á÷À» Àΰ£À̳ª ¿µÀå·ù¿¡ ÁÖÀÔÇÏ¸é µ¿ÀÏÇÑ º´ÀÌ ¹ß»ýÇÑ´Ù. ÇÁ¸®¿Â ´Ü¹é(PrP)Àº 1982³â Stanley PrusinerÀÇ ¿¬±¸½Ç¿¡¼ ¸é¾ç¶³¸²º´À» ÀüÆÄ½Ãų ¼ö ÀÖ´Â ¹°Áú·Î óÀ½ ¹ß°ßµÇ¾ú°í, ÀÌ ¹°ÁúÀÌ Á¤»óÀÎ ¥á-helix ¾ÆÇü(PrPc)¿¡¼ ¥â-pleated sheet ¾ÆÇü(PrPsc ȤÀº PrPres)À¸·Î ÀÔü±¸Á¶°¡ ¹Ù²î¸é proteinase K µî¿¡ ºÐÇØµÇÁö ¾Ê´Â °¨¿°·Â°ú Àü¿°¼ºÀ» °®´Â ¹°ÁúÀÌ µÈ´Ù. ÀÌ¿Í °°ÀÌ PrPsc´Â Á¤»ó¼¼Æ÷ÀÇ PrPc¿¡¼ Çü¼ºµÇ´Âµ¥ PrPscÀÇ Çü¼º¸ÞÄ¿´ÏÁòÀº ¾ÆÁ÷ ¹àÇôÁöÁö ¾Ê¾Ò´Ù. ÀÌ·± ÀÔü±¸Á¶ÀÇ º¯È´Â ÀÚ¿¬È÷ ¹ß»ýÇÏ´Â °æ¿ì¿¡´Â ¼Óµµ°¡ ¸Å¿ì ´À¸®°í, °¡Á··ÂÀ» °®´Â Áúȯ°ú °°ÀÌ À¯ÀüÀÚÀÇ º¯À̰¡ ÀÖ´Â °æ¿ì¿¡´Â ´õ ºü¸£°Ô »ý±æ ¼ö ÀÖ´Ù. »ç¶÷ÀÇ PrPc À¯ÀüÀÚÀÎ PRNP´Â 20¹ø ¿°»öüÀÇ ´Ü¿Ï¿¡ ÀÖ´Ù. ³ôÀº º¸Á¸°µµ¿¡µµ ºÒ±¸Çϰí ÇÑ Á¾¿¡¼ À¯·¡µÈ °¨¿°¹°ÁúÀÌ ´Ù¸¥ Á¾¿¡¼µµ Á¤»ó ´Ü¹é À¯ÀüÀÚ¸¦ °®°í ÀÖ´Â µ¿¹°¿¡´Â ½±°Ô °¨¿°µÈ´Ù. PRNP À¯ÀüÀÚ¸¦ ¾ø¾Ø µ¿¹°Àº PrPscÀÇ °¨¿°¿¡ ³»¼ºÀÌ ÀÖ´Â °ÍÀÌ °üÂûµÇ¾î¼ PrPscÀÇ °¨¿°¿¡ PRNP À¯ÀüÀÚ°¡ °ü¿©ÇÏ´Â °ÍÀ» ¾Ë ¼ö ÀÖ´Ù. ½Å°æÁ¶Á÷¿¡ PrPsc°¡ ÃàÀûµÇ¸é ÇÁ¸®¿Âº´ÀÌ ¹ß»ýÇÏ´Â °ÍÀ¸·Î »ý°¢ÇÏÁö¸¸ ½Å°æ¼¼Æ÷ÀÇ ¼Õ»óÀÌ ¹ß»ýÇÏ´Â ±âÀüÀº ¾ÆÁ÷µµ ±Ô¸íµÇÁö ¾Ê¾Ò´Ù. ÇÁ¸®¿Âº´Àº ´ëºÎºÐ »ê¹ß¼ºÀ¸·Î ¹ß»ýÇϰí ÀϺδ À¯Àüº´À̳ª Àü¿°º´°ú ºñ½ÁÇÑ ¹ßº´¾ç»óÀ» º¸ÀδÙ. À¯Àüº´ÀÎ °æ¿ì¿¡´Â PRNP À¯ÀüÀÚÀÇ ´Ù¾çÇÑ º¯À̰¡ °ü¿©Çϴµ¥, ÇöÀç±îÁö °¡Á··ÂÀ» °®´Â Å©·ÎÀÌÃ÷ÆçÆ®-¾ßÄߺ´°ú Ä¡¸íÀû °¡Á·ºÒ¸éÁõÀ» ÀÏÀ¸Å°´Â À¯ÀüÀÚÀÇ º¯È°¡ ¾Ë·ÁÁ³´Ù. ÇÁ¸®¿Âº´ÀÇ Àü¿°°æ·Î´Â PrPsc¿¡ ¿À¿°µÈ ¼ö¼ú ±â¼ö, ³úÆÄ°Ë»ç½ÃÀÇ Àü±Ø, °¢¸· µîÀÇ À̽ÄÀå±â ¶Ç´Â »çü·ÎºÎÅÍ Á¦Á¶µÈ ¼ºÀåÈ£¸£¸ó µîÀÌ´Ù. |
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| ¿µ¹® | senile dementia | ÇÑ±Û | ³ëÀÎÄ¡¸Å |
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| ¼³¸í | ³ëÈ¿¡ µû¸¥ ³úÀÇ ÅðÇ༺ º¯ÈÀÇ °á°ú ³ªÅ¸³ª´Â ³ëÀμº Á¤½ÅÀå¾Ö. ÁÖ·Î Áö´ÉÀÇ ±â´ÉÀúÇÏ·Î ÀϾ´Âµ¥, 65¼¼ ÀüÈĺÎÅÍ 70¼¼ÀÇ ³ë³â±â¿¡ ÀϾ´Â °æ¿ì¸¦ ¸»ÇÑ´Ù. »ý¸®ÀûÀÎ ³ëÀÎÄ¡¸ÅÀÇ ÀÌÇà¿¡ ´ëÇØ¼´Â Çö»óÀûÀ¸·Î ¸íÈ®ÇÑ ±¸º°À» ÇÒ ¼ö ¾ø´Ù. ³ëÀμº º¯È·Î´Â À°¾ÈÀ¸·Î´Â ƯÈ÷ À̸¶¿±¿¡¼ ÇöÀúÇÑ ³úÀÇ À§ÃàÀ» º¼ ¼ö ÀÖÀ¸¸ç, Á¶Á÷ÇÐÀûÀ¸·Î´Â ½Å°æ¼¼Æ÷ÀÇ °¨¼Ò, ½Å°æ¼¼Æ÷ ³»ÀÇ ¸®Æ÷ÇÁ½ºÄ£ Áõ°¡, ³ëÀιÝÀÇ ÃâÇö, ½Å°æ¿ø¼¶À¯ÀÇ ºñÈÄ µîÀ» µé ¼ö ÀÖ´Ù. Áõ¼¼·Î´Â ½ÉÇÑ °Ç¸ÁÁõ°ú ±â¾ïÀå¾Ö·Î ½ÃÀÛÇÏ¿© ±×·± Àå¾Ö¸¦ ¾ó¹ö¹«¸®±â À§ÇÑ ¸»¸¸µé±â ¶Ç´Â Áö°¢ÀúÇϰ¡ »ý±â°í, »ç°í¸é¿¡¼´Â ±º¼Ò¸®¸¦ ´Ã¾î³õ°Å³ª Àǿ常 ¾Õ¼¸é, ÆÇ´Ü·ÂÀ̳ª Ã߸®·ÂÀÌ ÀúÇϵǰí, »ý»êÀû »ç°í°¡ ¼Ò½ÇµÇ¸ç, °è»ê·ÂÀÌ ¼èÅðÇϰí, »çÅ¿¡ ´ëÇÑ ÆÇ´ÜÀÌ ºÒ°¡´ÉÇØÁö´Â µîÀÇ Áõ¼¼°¡ ³ªÅ¸³´Ù. |
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| ¿µ¹® | dementia | ÇÑ±Û | Ä¡¸Å |
|---|---|---|---|
| ¼³¸í | ³úÀÇ ±âÁúÀû Àå¾Ö¿¡ ÀÇÇÏ¿© ÈÄõÀûÀ¸·Î ÀϾ´Â ȸº¹ºÒ´ÉÀÇ Áö´ÉÀå¾Ö·Î¼, ÀǽÄÀå¾Ö¿¡ ÀÇÇÑ °ÍÀÌ ¾Æ´Ñ °ÍÀ» ¸»ÇÑ´Ù. Áö´É, ÀÇÁö, ±â¾ï µî Á¤½ÅÀûÀÎ ´É·ÂÀÌ µÎµå·¯Áö°Ô °¨ÅðÇÑ »óÅ·μ Á¤½Å¹Ú¾à°ú °°Àº Áö´É Àå¾ÖÀε¥, Á¤½Å¹Ú¾àÀÌ ÁÖ·Î Áö´É¹ßÀ°ÀÌ ÁöüµÇ°Å³ª Á¤ÁöµÈ °ÍÀÎ µ¥ ºñÇÏ¿© Ä¡¸Å´Â ÀÏ´Ü ¹ß´ÞµÈ Áö´ÉÀÌ ´ë³úÀÇ ÁúȯÀ¸·Î ÀÎÇØ Áö¼ÓÀûÀ¸·Î ÀúÇÏµÈ »óŶó´Â Á¡¿¡¼ ±¸º°µÈ´Ù. °ú°ÅÀÇ Ã¼Çè¿¡ ÀÇÇÑ ±â¾ïÀ̳ª Áö½ÄÀÌ ´ÜÆíÀûÀ¸·Î ³²¾Æ ÀÖ°í, ÀüüÀûÀ¸·Î ÀÏÁ¤ÇÏÁö ¾ÊÀº Áö´ÉÀå¾Ö°¡ ³ªÅ¸³´Ù. |
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| DP | data processing; deep pulse; definitive procedure; degradation product; degree of polymerization; de... |
|---|---|
| MID | maximum inhibiting dilution; mesioincisodistal; midinfarct dementia; minimum infective dose; minimum... |
| PRN | Physicians Research Network; polyradiculoneuropathy; prion |
| PRNP | prion protein |
| Prp | prion protein |
| PrP's | Prion Protein's |
|---|---|
| PrP(C) | Prion protein |
| PRNP | Prion protein gene |
| MID | 6-multiinfarct dementia |
| ADC | AIDS dementia complex |
| prion | The word, for proteinaceous infectious agent, was coined in 1982 by neurologist Stanley Prusiner as part of a hypothesis regarding ailments bearing aetiologic resemblance to those caused by slow viruses (for instance, kuru). The hypothesis has been borne out by investigation. Prions are now believed responsible for several transmissible neurodegenerative diseases Origin: proteinaceous infectious particle (05 Mar 2000) |
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| prion diseases | Transmissible and genetic neurodegenerative diseases of humans and animals caused by prions. The diseases are usually characterised by vacuolation in the gray matter and result in ataxia, motor disturbances, dementia, and progression to a fatal outcome. They include creutzfeldt-jakob syndrome, gerstmann-straussler syndrome, kuru, scrapie, fatal familial insomnia, bovine spongiform encephalopathy (encephalopathy, bovine spongiform), transmissible mink encephalopathy, and chronic wasting disease of mule deer and elk. The literature has sometimes referred to these as unconventional slow virus diseases. (12 Dec 1998) |
| prion protein | Small, infectious proteinaceous particle, of non-nucleic acid composition because of its resistance to nucleases; the causative agent, either on a sporadic, genetic, or infectious basis, of six neurodegenerative diseases in animals, and four in humans; the latter include the spongiform encephalopathies of kuru, Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome and fatal familial insomnia. The gene encoding for the PrP is found on chromosome 20. Synonym: prion. (05 Mar 2000) |
| AIDS dementia | <immunology> A frequent cerebral condition in people with AIDS that results in the loss of cognitive capacity, affecting the ability to function in a social or occupational setting. Its cause has not been determined exactly, but may result from HIV infection of cells in the brain or an inflammatory reaction to such infection. (09 Oct 1997) |
| AIDS dementia complex | <immunology> A frequent cerebral condition in people with AIDS that results in the loss of cognitive capacity, affecting the ability to function in a social or occupational setting. Its cause has not been determined exactly, but may result from HIV infection of cells in the brain or an inflammatory reaction to such infection. (09 Oct 1997) |
| Alzheimer's dementia | <disease> A progressive, neurodegenerative disease characterised by loss of function and death of nerve cells in several areas of the brain leading to loss of cognitive function such as memory and language. The cause of nerve cell death is unknown but the cells are recognised by the appearance of unusual helical protein filaments in the nerve cells (neurofibrillary tangles) and by degeneration in cortical regions of brain, especially frontal and temporal lobes. Alzheimer's disease is the most common cause of dementia. (22 May 1997) |
| vascular dementia | A state of diminished cognition that is the results from repeated cerebral strokes with a step-like deterioration in intellectual functions with focal neurological signs, as the result of multiple infarctions of the cerebral hemispheres. Synonym: multi-infarct dementia. (07 Mar 2000) |
| paralytic dementia | Dementia and paralysis resulting from a chronic syphilitic meningoencephalitis. Synonym: dementia paralytica. (05 Mar 2000) |
| catatonic dementia | Dementia with catatonic symptoms. (05 Mar 2000) |
| multi-infarct dementia | <neurology> This form of dementia is caused by a number of strokes in the brain. These strokes can cause specific symptoms, depending on their severity and location and can cause general symptoms of dementia. MID cannot be treated, once the nerve cells die, they cannot be replaced. However, the underlying condition leading to strokes (for example, high blood pressure, diabetes) can be treated, which may help prevent further damage. Synonym: vascular dementia. (22 May 1997) |
| presenile dementia | Dementia of Alzheimer's disease developing before age 65. Synonym: Alzheimer's disease. Primary dementia, dementia occurring independently as a mental disorder. (05 Mar 2000) |
| primary senile dementia | <disease> A progressive, neurodegenerative disease characterised by loss of function and death of nerve cells in several areas of the brain leading to loss of cognitive function such as memory and language. The cause of nerve cell death is unknown but the cells are recognised by the appearance of unusual helical protein filaments in the nerve cells (neurofibrillary tangles) and by degeneration in cortical regions of brain, especially frontal and temporal lobes. Alzheimer's disease is the most common cause of dementia. (22 May 1997) |
| secondary dementia | Chronic dementia following and due to a psychosis or some other underlying disease process. Senile dementia, dementia of Alzheimer's disease developing after age 65. Toxic dementia, dementia caused by an exogenous agent. (05 Mar 2000) |
| senile dementia | <neurology> A form of dementia caused by destruction (atrophy) of the frontal lobes of the brain. This condition leads to the progressive deterioration of mental functioning. Incidence: 9 out of 10,000 people in the population. (27 Sep 1997) |
| delirium, dementia, amnestic, cognitive disorders | Cognitive disorders including delirium, dementia, and other cognitive disorders. These may be the result of substance use, trauma, or other causes. (12 Dec 1998) |
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