Izifo Prion: izimbangela, izimpawu, uphethwe, ukuphathwa

izifo Prion - uhlobo olukhethekile izifo ezinzima neurodegenerative nezilwane. Basuke izimpawu ukulimala kobuchopho lesichubekako, ezimweni eziningi uphele lapho ngoba wayebona ukuthi usezokufa.

Yiziphi prions?

Lokhu amaprotheni isakhiwo ekhethekile. Zingaba kungokwemvelo futhi ingxenye izicubu abantu bephilile futhi izilwane, kanye okungavamile, okubangela izinhlobo ezahlukene izifo. Kuze emashumini ambalwa eminyaka edlule, kukholakala ukuthi isakhiwo eziphilayo kufanele libe okuthiwa acid nucleic - RNA ne-DNA. Siyabonga kubo, kungenzeka nokuzala. Amagciwane, isikhunta, izinyoni, izilwane "zakha" acid nucleic. Kuqala kwakukholelwa bazitshela ukuthi ukungabikho yabo ezicutshini akunakwenzeka nokuzala ngokugcwele. Prion kwamaprotheni kwakushintsha ngokuphelele la imibono.

Lezi ama-molecule enziwe kuphela amaprotheni, kodwa is zihlukaniswa ikhono nokuzala. Engena phakathi umzimba, babangela ushintsho ezikuyo ku prions evamile sokugembula, ngaleyo ndlela kwande isibalo sabo. Le nqubo idinga isikhathi eside kunalokho kabusha amagciwane noma amagciwane, ngakho esikhathini lokungena umzimba engqamuzaneni ekuthuthukiseni lesi sifo kuthathe iminyaka eminingana.

I izakhiwo prions

Prions nezifo prion zinezikhathi ukumelana okusezingeni eliphezulu. izindlela Iningi magciwane ayizwakali ekubhekaneni kubo. Prions kungukuthi libhujiswa abilayo, zingamelana abandayo kuze kube -40 degrees Celsius. Abanaso khombisa luvelo emisebeni ye-UV nokugcina impahla yabo ngesikhathi kucutshungulwa formalin.

Izici isakhiwo wamaprotheni molecule kuholela yokuthi umzimba womuntu awukwazi ukulwa nabo. Akayena uyakwazi kokukhiqiza amasosha omzimba ngokumelene prions, hhayi ehlasela lymphocyte yabo akuzange kubonakale uqaphela. Lokhu kusho ukuthi Ukungena zishintshe emzimbeni womuntu kubandakanya izindawo elivela kuzo isifo.

izifo Prion: umlando ukutholakala

Ngo-1982, Stenli Pruziner owokuqala owachaza lesi izifo prion, ngoba ngalo kamuva uMklomelo KaNobel.

Esikhathini eside ngaphambi kokuba ukutholakala kwabo, ososayensi be emsebenzini wabo wahlola eziningi pathologies womuntu nezilwane, okuyinto abangela isikhathi eside ayihlonzekanga. Ekhulwini XVIII e-United Kingdom liye bhalisiwe, "scrapie" imvu. Izilwane abaphethwe ukulunywa ezinzima, ukunyakaza iziyaluyalu kanye nokuquleka, okuyinto bakhomba CNS. Ngo-1957, uDaniel Carleton Gajdusek wachaza isifo esizweni, Fore kabani abantu ababehlala ezintabeni zasePapua New Guinea. I zokugula kwakuhambisana nezinga ubuzimu wadlula komunye umuntu kuya komunye.

Kusukela ngo-1986, e-England, futhi kamuva nakwamanye amazwe amaningi, ososayensi baye waloba nokuqubuka ezimbalwa kamuva unikezwa igama "ithuku ezinkomeni". It ogxile kuthinta izinkomo. "Mad inkomo isifo" emva esifushane sekufinyelele iqophelo eliphezulu, futhi isizathu nesenzakalo zakhona ziye zaba prions. Eminyakeni 90 ochwepheshe zikufakazela ukudluliswa kwalesi sifo kunendoda kanye ubisi inyama yezinkomo.

Okwamanje, ucwaningo enemininingwane nge izimbangela akubonakali izifo liye laba nomthelela yokuthi ososayensi baye benza eziningi iziphakamiso sokuthuthukiswa prion nemvelo. Phakathi kwazo kunane-pathology ka Creutzfeldt-Jakob isifo, isifo i-Alzheimer. Izimpawu kanye izimpawu zalezi izifo abe kuningi abafana ngakho. Naphezu kwentuthuko omkhulu kulolu cwaningo yalezi zinkinga, kuningi okusadingeka angaphezu eqondakalayo.

Wenzenjani ungenwe?

Ngo inqubo yokwelapha yesimanje, kunezindlela ezintathu lengculaza.

1. Athathelwana. Prions zidluliselwa zenye mammalian kwenye. okukhulunywe ngaphambilini ukuba khona okuthiwa kwezinhlobo isithiyo. Lokhu kusho ukuthi ukudluliswa kusuka inkomo akunakwenzeka indoda. Ososayensi ukuphikisa lo mbono. Amaphrotheni molecule angadluliselwa usuka emfuyweni egulayo noma wobuntu. Izimbangela zezifo prion zibangelwa ukungenisa inyama / itheleleke ubisi isilwane, ukusetshenziswa izicubu yemvelo (ukufakelwa corneal, imikhiqizo yegazi, nokunye. D.). biomaterials abahlukahlukene babe ngamazinga ahlukene pathogenicity. Okukhulu kunakho infectivity show ubuchopho izicubu, isinyathelo esilandelayo matasa igazi nemikhiqizo.
2. Ukubambezeleka. Lesi sifo eba khona ukuguquka kwezakhi zofuzo, abasafufusa chromosome 20. Kuyinto kule ndawo unesibopho khona amaprotheni evamile prion. Ukusebenza kwawo ayikaqondwa ezaziwayo. Endabeni zofuzo esikhundleni prion enempilo sokugembula kwemiqondo, okuyinto nakanjani kuholela ukuthuthukiswa izifo.
3. Okungatheni (nesenzakalo okuzenzakalelayo amaprotheni normal).

Ngakho, izifo prion kungaba kokubili uhlamvu zenani elikhulu eziyifa kanye ezithathelwanayo. Kungakhathaliseki indlela Ukungena amaprotheni normal emzimbeni, kungadaleka ukutheleleka kwabanye.

Yini eyenza prions emzimbeni?

amaprotheni okungavamile libhekene ikhono lazo kubangele spongiform encephalopathies, okusho CNS. Kusukela iphuzu morphological buka, lokhu kusho ukwakheka izimbotshana amangqamuzana obuchopho, ukufa neuronal, overgrowth endaweni yabo futhi ukuqeda zezinzwa izicubu yoma zobuchopho. Against isizinda kumiswa Amahlukuzo prion amyloid zingcwecwe uyalandelwa. Zonke lezi zinqubo zenzeka ngaphandle izimpawu ezisobala ukuvuvukala.

Yini izifo yilezi prion?

Kuze kube manje, ososayensi abakwazi igama ngokunembile izifo ezimbalwa, okuyizinto imbangela ukuqothuka kwezinwele okungavamile kuphelile amaprotheni izakhiwo:

• isifo Creutzfeldt-Jakob;
• isifo Kuru;
• Alpers (sithambile encephalopathy okuqhubekayo) isifo;
• ebulalayo ukuqwasha omkhaya;
• isifo Gerstmann-Shtreusslera-Scheinker.

Okulandelayo, sibheke ngamunye kabanzi egunjini lokuhlolwa kwezifo.

Creutzfeldt-Jakob isifo

Creutzfeldt-Jakob isifo libhekene ukwehlukana kwayo, ngakho ochwepheshe razdeleli ke amafomu eziningana:

• oqabukelayo;
• umndeni;
• iatrogenic;
• ifomu elisha atypical.

Okungatheni isifo samuntu ngaphambilini wacabangela luvame kakhulu. izimpawu yakhe yokuqala ukuvela ngemuva kweminyaka eyi-55. Nokho, kule minyaka eminyakeni embalwa izibalo ishintshile. Ngemva ukubukeka ulwazi Igagasi "ithuku ezinkomeni" ziya ziba mandla waqala ukurekhoda amacala amafomu atypical ngenxa izinkomo ukutheleleka. Ukuze lolu hlobo nobuntu ukubukeka ngaphambili. Ezimweni eziningi, intsha ekulesi simo. Izimpawu zihlukaniswe amaqembu amabili okunemibandela: zezinzwa zengqondo. Ekuqaleni, itheleleke ikhanda ngokuphawulekayo, ukuphazamiseka kokulala, kwehle iphango. Kancane kancane, lezi zimpawu ungeze inkumbulo ukungakhuli, ukulahlekelwa umbono. nokugula ngengqondo ziyabonakala ngesimo ukusangana kanye imicabango yokuzakhela. Lesi sifo libhekene ukuthuthukiswa ngokushesha esigabeni sokugcina libhekene baxhashazwe ephelele emzimbeni. Umuntu ulahlekelwa ukulawula umsebenzi kwezitho okhalo. Nge kokutshelwa ngokukhubazeka kwengane kubantu abahlala hhayi ningi than iminyaka emibili.

Ukubukeka ifomu ngofuzo kubangelwa ukuguquka kwezakhi zofuzo ezingeni isakhi sofuzo ezonini ye-chromosome 20. Lesi sifo libhekene i autosomal uhlamvu evelele. Izimpawu zokuqala zenzeka eminyakeni engaba ngu-5 ngaphambili kuka ngefomu oqabukelayo.

ifomu Iatrogenic eba ngenxa ukutheleleka womuntu phakathi nokuhlinzwa. Imininingwane yezibalo kulolu khetho akulona ukugula, ngoba kunzima ukuveza ubufakazi bokuthi pathogenesis izifo prion. Isikhathi bayashintshana ekufukameleni zokhahlamba kusuka 7 izinyanga kuya eminyakeni 12. Okunqumayo inhlanganisela yezici ezimbalwa: inqubo Ukungena amaprotheni normal emzimbeni, inombolo yabo, original genotype umuntu. Indlela esheshayo isifo ezikhulayo by Ukungena ngqo ka prions kwezicubu ubuchopho ngenxa ukuhlinzwa. Isikhathi esiningi iyadingeka ngesikhathi isifo isizinda ukufakelwa corneal noma laseDura Mater. Iziguli kancane kancane ataxia cerebellar, inkulumo kahle futhi imisipha ithoni, komqondo.

"Mad inkomo isifo" waqala ukuthola ukuhlobana ngemuva kwalesi sifo Izinkomo ngo-90s. isifo Prion, izimpawu ezivela abaneminyaka ephakathi kwengu-30 kuya eminyakeni engu-40, obulalayo kubantu. Njenganoma ifomu iatrogenic, izimpawu zokungasebenzi kahle kwemizwa yibona ngokwengqondo.

Fatal ukuqwasha ngofuzo

Lesi sifo kuyinto autosomal uhlamvu evelele, okuyinto ngocansi kuphela ngokulandelana. ukuqwasha Obulalayo akuvamile. Yena e nesayensi kusukela ngo-1986. Izimpawu zayo kuqala abaneminyaka ephakathi kwengu-25 kuya eminyakeni engaba ngu-71.

Epidemiology izifo prion yalolu hlobo kuthiwa insufficiently uhlole. Uphawu oyinhloko ebulalayo ukuqwasha ngofuzo kuyinto nokungalali. Umzimba kancane kancane ilahlekelwa amandla ayo ukuze ulungise ngokugcwele esigabeni sihlale siphapheme futhi ubuthongo. Futhi, iziguli zivele ukunyakaza iziyaluyalu futhi ukuba buthakathaka kwezicubu. Sekube amacala iziyaluyalu autonomic ukuthi manifest anda blood pressure, ukujuluka ngokweqile. Of nokugula ngengqondo kungaba kuqashelwe uvalo, ukuhhema ezibukwayo, neziqephu ngakanye ukudideka. Ngenxa ukuqwasha ukukhathala njalo kwenzeka, isiguli uyafa.

Kuru

ifomu prions ezithathelwanayo abangu wafunda ngokuningiliziwe ngoba lesi sifo, ikakhulukazi yesizwe amazimuzimu. Kuze 1956 phakathi izakhamuzi Papua - New Guinea Kwasakazwa okuthiwa isiko ubuzimu isiko - ukudla ubuchopho womuntu ohlongakeleko. Kukholakala ukuthi omunye amalungu alesi sizwe kwavela emagciwaneni, kamuva wasakazekela kwezinye abantu emva isiko. Kusukela lapho kwesulwa le isiko amacala isifo aye alotshwa kulo izikhathi eziningana kancane njalo namuhla lesi sifo cishe ingekho.

Isikhathi bayashintshana ekufukameleni zokhahlamba kusuka eminyakeni 5 kuya ku-30. Yingakho lesi sifo Kuru ukubizwa ngokuthi 'kancane virus ukutheleleka "isigaba. Nokugula iziyaluyalu cerebellar wabonakaliswa ngokuhleka zingalawuleki, ukugwinya ukungasebenzi kanye buthakathaka kwezicubu. Ezigabeni ukuphela ekuthuthukiseni komqondo. Abantu nale Ihlahlubo baphile akukho kuka izinyanga ezingu-30.

Alpers isifo

Lesi sifo ikakhulukazi kwenzeka ezinganeni ezincane (eneminyaka engaphansi kwengu-18). Ukugula idluliselwa i autosomal ngendlela esikhubazayo, esimweni kuqondana ezimbili pathogenic izakhi zofuzo obaba nomama. Phakathi izimpawu eziyinhloko bungabonakala angaboni kahle futhi nokuquleka. Ngo yezokwelapha izincwadi zikhona izincazelo ukugula acute okwenzeka ngo-stroke hlobo. Alpers sifo futhi libhekene izilonda kwesibindi, okuyinto ngokushesha esiholela ukusha kwesibindi kohlobo ezingamahlalakhona ziphethe isifo sokusha. Iziguli bayafa ngenxa ukudakwa izinyanga 12 emva ukuxilongwa izimpawu zokuqala.

Gerstmann syndrome-Shtreusslera-Scheinker

Lolu khetho is uphakathi lezinceku isifo hlobo. ezingavamile kakhulu (enye icala abantu abangaba yizigidi ezingu-10). Izimpawu zokuqala ngokuvamile kubonakala iziguli ngaphezu kweminyaka engu-40. Ukuthuthukiswa lesi sifo iqala iziyaluyalu cerebellar. Ekuqaleni isiyezi livela. Njengoba isifo siqhubeka ukwephulwa koordinatornye kancane kancane ukunyakaza ezimele, kusuka kube nzima. Kanye izimpawu ezibalwe avele iziyaluyalu imisipha ithoni, ukwehla umbono nokuzwa, izinkinga ne ukugwinya nasekukhuleni nokuzala. Ngo ekupheleni-esiteji fixed odokotela kuwukubonakaliswa komqondo. Isilinganiso sobude bokuphila iziguli nale Ihlahlubo kuze kube iminyaka engu-10.

Alzheimer futhi isifo i-Parkinson

Alzheimer futhi isifo i-Parkinson, izimpawu kanye izimpawu ezithinta imvelo langempela, ekuthuthukiseni ngendlela efanayo ukuze prion izifo. Ama-molecule ye-beta amyloid, tau amaprotheni nezinye izakhiwo lwakha ezifakwa bemvelo pathogenic kwezicubu ingqondo. Nokho, ukubamba lezi izifo engenakwenzeka. Lokhu kusho ukuthi fibrils amyloid akhiwa obonakele amaprotheni amangqamuzana, kodwa isenzo "enempilo" "ogulayo" awusebenzi.

Muva nje, ososayensi baye baqhuba uchungechunge izifundo in amagundane, okuyinto wakuphika lokhu nokucabanga. Ngemva isingeniso amaprotheni pathogenic ebuchosheni nhlobo Isilwane esiphilile anakho isici amyloid zingcwecwe. Lokhu kusho ukuthi amaprotheni lwegciwane usengakwazi ngegciwane isakhiwo enempilo. Ukutholakala kungokwalabo Ochwepheshe kusukela University of Texas. Esikhathini esizayo esiseduze kuyofika omunye umsebenzi ososayensi waseLondon, okuyinto ebonisa ukuthi Alzheimer isifo, izimpawu kanye izimpawu zokugula kungaba ngokugcwele ingadlulela kusuka komunye umuntu.

Khumbula ukuthi isifo i-Parkinson libhekene ukulahlekelwa okuqhubekayo izinzwa ezikhiqiza Dopamine, a neurotransmitter. Ngenxa yalokhu, umuntu isiqondiso okhathazekile of ukunyakaza kanye tonus kwemisipha, okuyinto livezwa liqhaqhazela, ukuqina jikelele. Parkinsonism uhlushwa wonke umuntu wokuhamba owaphenduka ayisithupha phesheya. Lesi sifo iqala ukuthuthukiswa yayo nge ngokunensa, okuyinto kwaphawuleka ngokukhethekile lapho umuntu ugqoka noma ithatha ukudla. Ngemva kwalokho, uma behlukaniswa inkulumo, ukugwinya ingqondo. Ngeshwa, umuthi kungenzeka ukuncoma ikhambi eliphumelelayo abantu kwathiwa unesifo "Parkinson". Izimpawu zalesi sifo kungenziwa mitigated by yokwelapha lingaze. Nokho, iningi lalezi izidakamizwa kubangela eziningi engemihle.

Alzheimer - isifo libhekene lilokhu libuna sezinzwa, okubangela iziguli ziba ukuwohloka komqondo. Izimpawu zokuqala zalesi sifo zenzeke njengoba ekuseni njengoba engu-40 ubudala. It sitholakale ezimweni eziningi abafundisekile kahle abantu. Umuntu nge izinga eliphezulu intelligence ithuba ezingcono ozicabangayo zokubhekana izimpawu Alzheimer ngenxa uxhumano eziningi phakathi izinzwa.

Lesi sifo iqala ukuthuthukiswa yayo nge inkumbulo iziyaluyalu. Isigaba esiyinhloko ngokuvamile singanakwa kwabanye. Izimpawu zokuqala zivame uzama ukufihla noma kusolwa ukucindezeleka futhi umsebenzi ngokweqile emsebenzini. Njengoba ukuqhubeka isithombe emitholampilo ye iyaguquguquka isifo. Isiguli is ayisekho eMpumalanga ngokwabo emkhathini, kusukela inkumbulo yakhe iconsi amakhono atholwe ngaphambili sokuloba, ukufunda. Okokuqala, ukhohlwe umcimbi olandelayo ngesikhathi. Lapho zokugula iqala ukuba bathuthuke, kubalulekile ukusebenzisa wonke amathuba ukuze silondoloze ikhono lomuntu ukuze self-service, ukuze bazame ukuvimbela okuvela kuyo ukucindezeleka. Isixazululo sale nkinga kungakusiza namandla kakhulu kuzwa noma izibuko afakwe kahle. ukuphathwa eqondile Alzheimer syndrome alikho. Lapho izimpawu eziyinhloko kubalulekile ukuthola luhlolo luphelele ngokubuyekeza isazi sezinzwa. ukwelapha ochwepheshe Ngokuvamile zitusa izidakamizwa lesi sifo ukuba lula futhi okwakheka ukuthuthukiswa yayo.

Ukuxilongwa izifo prion

izinyathelo Specific zokuxilonga Okwamanje emelelwa. Ngokwesibonelo, okufanayo Bheka okutholakele ku Creutzfeldt-Jakob isifo, kukhona ngezinye pathologies ingqondo. It libhekene ongaphakeme MRI ukubaluleka kokuthola inkinga, ngoba u-80% eziguli zembula amasignali non-ethize. Nokho, lolu cwaningo Kusenza sikwazi ukuzwa ukuthi ubuchopho yoma. namandla aso sibe sibi njengoba intuthuko izifo prion womuntu.

Imiphumela yaloku kuhlola okungenhla umehluko Kwenziwa yonke pathologies, loluvetwa okuyinto komqondo (Alzheimer isifo, vasculitis, neurosyphilis, herpes encephalitis nabanye).

Ukwelashwa izindlela

Ngeshwa, esikhathini sanje, zonke izifo prion azinakulungiswa. Iziguli babelwe yokwelapha lingaze nge anticonvulsants, okuyinto kunciphisa ukuhlupheka kuphela. Isibikezelo esidumazayo. Zonke izifo prion eyaziwa ziyabulala kakhulu nemvelo.

Okwamanje, ososayensi ezivela emhlabeni wonke abakhuthalele ukusesha imithi jikelele. Ucwaningo olwenziwe usebenzisa izilwane. Kusolwa ukuthi ekulweni izifo ezifana stem cells izosetshenziswa kamuva, kanye nemvubelo ezivamile. izidakamizwa kokulinganisa Okwamanje awunawo abe nekghono eliphezulu, ngakho inhloso yabo kubhekwa okungalungile.

izinyathelo

Kusukela ukuthuthukiswa ezahlukeneyo oqabukelayo futhi ngofuzo izifo prion ukuze bazivikele cishe akunakwenzeka. Ezinye izifo luqedwe ngu ngidlula ukuhlolwa kokufaneleka okukhethekile zofuzo. Nokho, kuyinto ezweni lethu kunzima kakhulu, ngoba elabhorethri enza lolu hlobo uphethwe, abangazazi phesheya.

Endabeni izifo lezinceku ngaphambi kokukhulelwa belulekwa ukuba ubonane nodokotela-sezakhi zofuzo. Lokhu kuzosiza sigweme izinkinga zesikhathi esizayo ne impilo yengane.

Ukuze bazivikele Creutzfeldt-Jakob isifo, kutuswa ukuba bangayeki ukudla inyama kusukela ezifundeni lapho ezifo izinkomo eqoshiwe. Okokuqala sikhuluma amazwe aseYurophu. Kufanele akufanele isetshenziswe ekwelapheni amalungiselelo enziwe kusukela egazini lika izilwane noma abantu. Kungcono ukuba esikhundleni saso kungene zokwenziwa analogue.

Prion izifo - akuyona ngokwanele wafunda izinhlobo izilonda ezithathelwanayo futhi esibangela ukuba zenzeka emzimbeni womuntu ku isizinda Ukungena amaprotheni ongaphilile. Ezimweni eziningi, ezisithintayo isimiso sezinzwa esiyinhloko. Isithombe emitholampilo libhekene nezimpawu ezifanayo. Ekuqaleni, umuntu, zaphelelwa uthando lokudla kanye umbono, siyabhubha ukubambisana emkhathini. Ngesikhathi esigabeni sokugcina ekuthuthukiseni komqondo uma isiguli ayikwazi ngokuzimela ukuzinakekela. Umphumela wanoma isiphi isifo ihlale ifana - ukufa. Okwamanje, odokotela abanalo usizo oluyilo ezinkantolo ngokumelene pathologies walolu hlobo.