Izifo Prion: oonobangela, iimpawu, isifo, unyango

izifo Prion - luhlobo olulodwa kwezifo ezinzima neurodegenerative abantu kunye nezilwanyana. Bona luphawulwa ukonakala kwengqondo zenkqubela phambili, kwiimeko ezininzi kuphela kunye kufa.

Yintoni prions?

Esi sakhiwo protein okhethekileyo. Iiplastiki zombini eziqhelekileyo kunye inxalenye nezihlunu abantu esempilweni nezilwanyana, kwakunye ezingaqhelekanga, ibangela iindidi ngeendidi zezifo. De kwiminyaka embalwa eyadlulayo, kwakukholelwa ukuba isakhiwo ophilileyo kufuneka siqulathe asidi ekuthiwa-acid - RNA ne-DNA. Enkosi kubo, kunokwenzeka ukuba nokuzala. Virus, nomngundo, iintaka, izilwanyana "ziquka" asidi acid. Ngaphambili bekucingelwa ukuba ukungabikho yabo kwizihlunu akunakwenzeka ukuba ande ngokupheleleyo. iiproteni Prion babuyele ngokupheleleyo ezi ngcamango.

Ezi molecule enziwe kuphela ngakwicala leeprothini, kodwa azakubalulwa ubuchule ukuzala. Zingene ephilayo, bakufikise inguqu equlathwe apha kule prions eqhelekileyo lwezidumbu, ukuze kwandiswe inani labo. Le nkqubo ifuna ixesha ngaphezu enomfanekiso iintsholongwane okanye iintsholongwane, ngoko ukususela ngethuba engena umzimba molecule kuphuhliso sifo kungathatha iminyaka emininzi.

Iimpawu prions

Prions kunye nezifo prion luphawulwa ukuxhathisa eliphezulu. iindlela Uninzi kweentsholongwane olungasebenziyo zokujongana nabo. Prions ukuba ibhujiswe ngokuxuba, unako ukumelana abandayo ukuya ku -40 degrees Celsius. Bona zingabonakaliyo uvakalelo imitha yelanga nokugcina iimpawu zazo ngexesha kuqhutyekekwe formalin.

Iimpawu ngesakhiwo zeprotheyini neemolekyuli kukhokelela ekubeni umzimba womntu awukwazi ukulwa nabo. Yena esikwaziyo ukuvelisa elilwa prions, hayi ngokuhlasela akazange ibonakala lymphocyte zabo ukuze babone. Oku kuthetha ukuba kwii-iimolekyuli emzimbeni womntu iquka isenzeko sisifo.

izifo Prion: imbali amazwe

Ngowe-1982, Stenli Pruziner kuqala wachaza izifo prion, kuba ngawo kamva imbasa Prize Nobel.

Kudala phambi kokuba ifunyanwe, izazinzulu kumsebenzi wabo Lwavavanya inani ezimbi zabantu kunye nezilwanyana, obe ubaqhiphukisa ixesha elide ayikwazanga ukuqinisekiswa. Kwinkulungwane XVIII kwi United Kingdom sele libhalisiwe, "scrapie" iigusha. Izilwanyana abaphethwe ukurhawuzelela kakhulu, ukuphazamiseka intshukumo nokuxutha izinto, nto leyo yakhomba CNS. Ngowe-1957, Daniyeli Carleton Gajdusek uchaze sifo Sobala isizwe, ogama abantu ababehlala kwiinduli zasePapua New Guinea. Le ngezifo kwakuhambisana inyama badlula ukusuka komnye umntu iye komnye.

Ukususela ngo-1986, eNgilani, yaye kamva kwamanye amazwe amaninzi, izazinzulu ziye urekhode loqhambuko ezimbalwa kamva wanikwa igama "ithuku". Oku ikakhulu kuchaphazela iinkomo. "Ithuku Mad" emva kwexesha elifutshane ixesha lifikelele etyhutyha kwaye isizathu ukwenzeka zawo prions. Kwiminyaka 90 Iingcali zingqineke ukudluliselwa kwesi sifo ebantwini kunye ubisi nenyama iinkomo.

Okwangoku, ufundo-nzulu kunye oonobangela akubonakali kwezifo kuye negalelo yokuba izazinzulu ziye zenza inani iziphakamiso uphuhliso prion indalo. Phakathi kwawo ke ngezifo lwesifo Creutzfeldt-Jakob, isifo i-Alzheimer. Iimpawu kunye neempawu zezi zigulo banezinto ezininzi abafana ngazo. Nangona inkqubela enkulu isifundo kwezi ukuphazamiseka, kusekuninzi ngaphezu ukuyiqonda.

wena bangenwe njani na?

Xa yesilungu, kukho iindlela ezintathu usulelo.

1. Ukubasulela. Prions zidluliselwa zenye mammalian kwenye. Ngaphambili wakhankanya ubukho ekuthiwa-umqobo iintlobo. Oku kuthetha ukuba ukudluliswa kusuka inkomo akwenzeki emntwini. Izazinzulu aphikise lo mbono. iimolekyuli protein yosulela ukusuka sisilwanyana esinesi sifo uyosuleleka okanye abantu. Izinto ezibangela izifo prion zibangelwa nokudla ukudla / ubisi abosulelekileyo isilwanyana, ukusetyenziswa inyama eziphilayo (abohlukeneyo kwenwebu yamehlo, iimveliso zegazi, njalo njalo. D.). biomaterials ezahlukene izidanga ezahlukeneyo phathogenicity. Le infectivity show lolwabo omkhulu ingqondo, inyathelo elilandelayo ahlala igazi kunye negazi iimveliso.
2. Njengelifa. Esi sifo senzeka phambi kwiDNA, asakhulayo chromosome yama-20. Yiyo le ndawo unoxanduva ubukho kwiproteni prion yesiqhelo. umsebenzi wayo ayikaqondwa yaziwa. Kwimeko yemfuza gene endaweni prion esempilweni kuhlelwe lwezidumbu, nto leyo ekugqibeleni kukhokelela kuphuhliso izifo.
3. Manqapha (isahlakalo yezizwe protini engaqhelekanga).

Ngenxa yoko, izifo prion kunokuba zombini ubhalo amafa esosulelayo. Kungakhathaliseki indlela kwii-protein ezingaqhelekanga emzimbeni, oko kungadala usulelo kwabanye.

Yintoni ebangela prions emzimbeni?

iiproteni ezingahambi luphawulwa ubuchule babo ukuba encephalopathies Spongiform, ie CNS. Ukusuka kwindawo morphological lwembono, oku kuthetha ukuyilwa ukuqanda iiseli zengqondo, ukufa neuronal, imithana endaweni yazo neziphela nokuqina isicwili yoma ebuchotsheni. Ngokuchasene nentsukaphi ukuyilwa izihloko umonakalo prion amyloid waphawula. Zonke ezi nkqubo lwenzeka ngaphandle iimpawu ezicacileyo ngenxa yokukrala.

Ziziphi izifo prion?

Ukuza kuthi ga ngoku, izazinzulu ngokuchanileyo sibala nje izifo ezimbalwa, ezo unobangela izakhiwo protein engaqhelekanga:

• isifo Creutzfeldt-Jakob;
• isifo kuru;
• Alpers (nomsi- Encephalopathy okuqhubekayo) izifo;
• ugwayi kwiintsapho ebulalayo;
• sifo Gerstmann-Shtreusslera-Scheinker.

Okulandelayo, khangela nganye iinkcukacha ngakumbi ngezifo.

isifo Creutzfeldt-Jakob

isifo Creutzfeldt-Jakob luphawulwa ukwahluka layo, ngoko iingcali razdeleli oko ngeendlela ezininzi:

• khona;
• umthetho wekhaya elithile;
• iatrogenic;
• ifom entsha Nedlac.

ngobuwena isifo manqapha ngaphambili ingqalelo kakhulu. zimpawu yokuqala ukuvela emva kweminyaka engama-55. Noko ke, kule minyaka imbalwa manani yokugqibela itshintshile. Emva kokuba imbonakalo ingcaciso bhubhani "ithuku" nalapha ngaphakatyhi waqalisa ukubhala iimeko Nedlac ngenxa usulelo iinkomo. Ngokuba lo hlobo uphawu imbonakalo ngaphambili. Kwiimeko ezininzi, abantu abaselula ubunzima. Iimpawu bohlulwa babe ngamaqela amabini enemiqathango: luvo nasengqondweni. Ekuqaleni, wathelela intloko ebuhlungu esiphawuliweyo, ukuphazamiseka ekulaleni, yehle onombilini. Ngokuthe ngcembe, ezi mpawu wongezelela memory lomonakalo, ukulahlekelwa umbono. abagula ngengqondo ziyabonakala ngokohlobo nemibono kunye iimfeketho zokufeketha. Esi sifo sizibonakalisa luphuhliso olukhawulezayo kwinqanaba lokugqibela luphawulwa nokungakwazi ngokupheleleyo emzimbeni. Umntu ulahlekelwa ulawulo phezu umsebenzi amalungu zangasese. Unesifo enjalo ngabantu abahlala akukho ngaphezu kweminyaka emibini.

Ukubonakala ifomu kwiintsapho kubangelwa ukuguquka kweli kwinqanaba sofuzo kwindawo le chromosome yama-20. Esi sifo ebalasele autosomal uphawu lubalaseleyo. Imiqondiso yokuqala kwenzeka malunga 5 kwiminyaka ngaphambili kunokuba uhlobo khona.

ifomu Iatrogenic ikhula ngenxa usulelo yabantu ngexesha utyando. ulwazi lwezibalo ukuba olu khetho alukho sisigulo, ekubeni kunzima ukuba ubungqina Pathogenesis izifo prion. Ithuba elisuke isusela ukusuka kwiinyanga-7 ukuya kweli-12. Ixhomekeka intlanganisela yezinto ezininzi: inkqubo elalana kweziyi ezingaqhelekanga emzimbeni, ngokwenani labo, kweejini umntu wokuqala. Isifo ezanda yi umthondo ngqo prions e isicwili sobuchopho ngenxa uqhaqho. Kufuneka ixesha elininzi ngethuba kwezifo imvelaphi abohlukeneyo kwenwebu okanye laseDura mater. Izigulane kancane kancane Ataxia cerebellar, intetho-nkene kwezihlunu, sengqondo esixhalabisayo.

"Ithuku Mad" waqala ukufumana ngokufaneleka emva kokuba ubhubhane kwi iinkomo kwi 90. isifo Prion, iimpawu ezivelayo phakathi kweminyaka eli-30 ukuya kwiminyaka ezimalunga nama-40, obulalayo ebantwini. Njengoko enendlela iatrogenic, iimpawu luvo ngabona ngokwengqondo.

Fatal ugwayi kwiintsapho

Esi sifo i autosomal ubhalo elibalaseleyo, nto leyo sisasazwa kuphela ngokulandelelana. ugwayi Fatal inqabile. Waziwa kwisayensi ukususela 1986. iimpawu zayo yokuqala phakathi kweminyaka eli-25 ukuya malunga 71 kulo nyaka.

Epidemiology izifo prion elolu hlobo ngokwaneleyo ekhangelwe nkalwana zonke. Uphawu ezingundoqo ukuphuthelwa esibulalayo kwiintsapho yi nokungalali. ngcembe umzimba uphelelwa ngamandla ayo ukunyenyisa ngokupheleleyo kwisigaba uphaphama kunye nokulala. Kwakhona, izigulane kuvela iingxaki intshukumo kunye nobuthathaka kwezihlunu. Kukho amatyala ukuphazamiseka yawo ezibonakalisa ukwanda uxinzelelo lwegazi, ukubila ngokugqithiseleyo. Ukuphazamiseka kwengqondo onokubonwa uphaphazele, nemibono ezibonwayo, kunye neziqephu ngakanye ukudideka. Ngokuba kwenzeka ukuphathwa rhoqo ukudinwa, isigulane uyafa.

Kuru

ifomu prions ezisulelayo zaye ifundwa iinkcukacha ngenxa yesi sifo, ingakumbi, wesizwe enzela. De 1956 phakathi kwabahlali zasePapua - New Guinea zasasazwa ekuthiwa isithethe mu wezithethe - besidla ingqondo yomntu oswelekileyo. Kukholelwa ukuba elinye lamalungu le ndlu kwavela usuleleko, leyo kamva yandele kwamanye abantu emva yezithethe. Ukususela kurhoxiswe eli siko amatyala izifo ebhalwe izihlandlo eziliqela alufane namhlanje esi sifo ngokwenza lungenzeki.

Ithuba elisuke isusela ukusuka kwiminyaka-5 ukuya kwi-30. Kungenxa yoko le nto sisifo Kuru ubizwa ngokuba "slow usulelo ntsholongwane" didi. Uyagula izifo cerebellar walibonakalisa ngokuhleka lawuleki, yokuginya kwekhono kunye izihlunu buthathaka. Kumanqanaba yesiphelo yokuvela sengqondo esixhalabisayo. Abantu nalo unesifo bangabi ngaphezu kweenyanga ezingama-30.

isifo Alpers

Isifo ubukhulu becala kwenzeka kubantwana abancinane (abangaphantsi kweminyaka eli-18). Ukugula ezidluliselwa ngendlela sofuzo autosomal, kwimeko kuqondana yemfuza zooyise ezimbini yayintsholongwane eyosulela kunye noomama. Phakathi iimpawu eziphambili ziyakwazi ukuchongeka ukungaboni kunye nokuxutha izinto. Zonyango iincwadi reference kukho iinkcazelo zokugula etsolo kwenzeka ngohlobo stroke. isifo Alpers linophawu izitshanguba kwesibindi, leyo iphuhlise ngokukhawuleza kohlobo nesiphelo kunye kwesibindi. Izigulane bayasweleka ngenxa ukunxila okanye iinyanga ezili-12 emva kokuba lokufunyaniswa iimpawu kuqala.

Gerstmann syndrome-Shtreusslera-Scheinker

olu khetho zichongwe uhlobo lwezifo njengelifa. inqabile kakhulu (icala elinye malunga-10 lezigidi zabantu). Imiqondiso yokuqala ngokuqhelekileyo kubonakala izigulane kwiminyaka engama-40. Uphuhliso onaso iqala nokuphazamiseka cerebellar. Ekuqaleni nesiyezi ibonakala. Njengoko esi sifo ukunyhashwa koordinatornye ngcembe intshukumo ezimeleyo kube nzima. Sikunye iimpawu zidweliswe kuvela ukuphazamiseka ithoni kwezihlunu, ukuncipha embonweni nokuva, iingxaki yokuginya nokuzala. Ekupheleni-nqanaba esisigxina oogqirha izenzo dementia. Iminyaka yokuphila kwezigulane lo unesifo ukuya kwiminyaka-10.

isifo i-Alzheimer kunye nesifo Parkinson kaThixo

isifo i-Alzheimer kunye nesifo Parkinson kaThixo, iimpawu kunye nemiqondiso leyo enemo eqhelekileyo, nokuphuhlisa ngendlela efanayo ukuba prion izigulo. Molecule beta amyloid, iiproteins otau kunye nezinye izakhiwo lwakha iidipozithi yendalo yayintsholongwane eyosulela kakhulu isicwili sobuchopho. Noko ke, ukuba ukubamba ezi zigulo akunakwenzeka. Oku kuthetha ukuba fibrils amyloid akhiwa iimolekyuli iiproteins eyonakeleyo, kodwa inyathelo 'uphilile "" abagulayo "alisebenzi.

Kutshanje, izazinzulu ziye wayeqhuba kuthotho lwezifundo zophononongo e iimpuku, okuphikayo le vekeleke xona. Emva kokuba kuqaliswe kwiiproteni yayintsholongwane eyosulela kwi ingqondo ngokupheleleyo isilwanyana ezingenabungozi unalo umonakalo amyloid isici. Oku kuthetha ukuba kwiproteni pathogen luyakwazi ukosulela isakhiwo esempilweni. Ukufunyanwa bobabo bezifo e University of eTexas. Kwixa elizayo uya kuza omnye umsebenzi wenzululwazi ukusuka eLondon, nto leyo ebonisa ukuba i-Alzheimer izifo, iimpawu kunye iimpawu zokugula unako ngokupheleleyo ukuba kudluliswe ukusuka komnye umntu ukuya komnye.

Khumbula ukuba izifo Parkinson kaThixo luphawulwa ilahleko okuqhubekayo luvo ezivelisa dopamine, a neurotransmitter. Ngenxa yoko, loo mntu ulawulo aphazamiseke iintshukumo kunye ufe dystrophy, apho kuboniswa lafa, ukuqina ngokubanzi. Parkinsonism unale wonke umntu wamakhulu, ubuyeleyo amathandathu amazwe. Isifo siqala Uphuhliso yalo motion kade, nto leyo oqaphelekayo ingakumbi xa umntu ofaka okanye uthatha isidlo. Kamva, kwaphulwa intetho, ukuginya ingqondo. Ngelishwa, linokungasebenzi iyeza singacebisi unyango olusebenzayo kubantu unesifo "Parkinson kaThixo". Iimpawu kunye iimpawu zesi sifo kuthomalaliswa unyango nomqondiso. Noko ke, uninzi kula machiza ebangela inani yeziphumo ebezingalindelekanga.

isifo i-Alzheimer - sisifo yayiphawuleka Ukubuna zemithambo-luvo, ebangela izigulane ukuphuhlisa sengqondo esixhalabisayo. Iimpawu sokuqala sale sifo kwenzeke kwangoko wayeneminyaka engama-40. It unesifo kwiimeko kakubi abafundileyo abantu. Umntu ithuba elingcono ukumelana iimpawu-Alzheimer ngenxa unxulumano ezininzi phakathi luvo inqanaba eliphezulu ubukrelekrele.

Isifo siqala Uphuhliso yalo memory iingxaki. Inqanaba lokuqala kudla azithathelwa ngqalelo kwabanye. Iimpawu zokuqala badla uzama ukufihla okanye lunokusolwa uxinezeleko omninzi kakhulu emsebenzini. Njengoko ukuqhubela phambili kumfanekiso kwezonyango eziguqulayo sifo. Le isigulane engasekho hlengisa ngokwabo emajukujukwini, ukususela memory drop lwakhe nezakhono ezifunyenwe ngaphambili sokubhala, ukufunda. Okokuqala, bakhohlwe isiganeko elilandelayo kwangexesha. Xa ngezifo eqalisa ukuba inkqubela, kuyimfuneko ukusebenzisa naliphi na ithuba ukugcina ubuchule bomntu lokuzimela-nkonzo, ukuzama ukuthintela ukwenzeka ngokudandatheka. Isisombululo sale ngxaki kunceda yokuncedisa ukuva enamandla ngakumbi okanye iiglasi ifakwe kakuhle. unyango oluthile lwe-Alzheimer syndrome ayikho. Xa iimpawu eziphambili kubalulekile ukufumana uviwo epheleleyo ngumntu luvo. ngokuba unyango leengcali ngokuqhelekileyo isincomo iziyobisi ukuba esi sifo ukubhexesha baninyanzela lwalo lokukhawulelana nophuhliso.

Uxilongo kwezifo prion

amanyathelo ezithile zokuxilonga nje okwangoku zimelwe. Umzekelo, iziphumo EEG efanayo njengoko izifo Creutzfeldt-Jakob, ezihleli nezinye izinto ezimbi ebuchotsheni. It lubonakala eliphantsi MRI ukubaluleka diagnostic, ukususela 80% ezigulane ityhila Imiqondiso non-ngqo. Noko ke, le sifundo kuvumela ukuba ukuchonga yoma ebuchotsheni. namandla aso sibi nenkqubela lwezifo prion kwabantu.

Ukuxilongwa ngokungafaniyo kwenzelwa zonke iintsholongwane, esinye sezibonakaliso zezifo ezibangelwa yingqondo (i-Alzheimer's disease, vasculitis, neurosyphilis, encephalitis yakhe kunye nezinye).

Iindlela zokwenza unyango

Ngelishwa, okwangoku zonke izifo ze-prion aziphilwanga. Izigulane zinqunywe unyango olusisigxina ngokusebenzisa i-anticonbulsants, okunciphisa uxinzelelo. Isibikezelo sidimazekile. Zonke izifo ezaziwayo zendalo ye-prion ziyabhubhisa abantu.

Ngoku iinzululwazi ezivela kwihlabathi lonke zifuna uphando lwamayeza jikelele. Izifundo zenziwa ngezilwanyana. Kucingelwa ukuba i-cell stem, kunye neyesambuko eqhelekileyo, iya kusetyenziselwa ukulwa nezi zifo. Amalungiselelo okucwangcisa okwangoku asebenzi kakuhle, ngoko ke injongo yabo ithathwa njengento engabalulekanga.

Amanyathelo okukhusela

Ukususela ekuphuhlisweni kweentsholongwane zesiphelo kunye nezizukulwana zezifo ze-prion, akunakwenzeka ukuba uzikhusele. Ezinye iintsholongwane zingabandakanywa ngokwenza uviwo oluyimfuza. Nangona kunjalo, eli lizwe lilukhuni kakhulu, kuba ii-laboratories ezenza lolu hlobo lokuxilonga zivela kwamanye amazwe.

Kwimeko yezifo eziza kuzalwa ngaphambi kokukhulelwa, kucetyiswa ukuba kudibaniswane nomzimba. Oku kuya kunceda kwikamva ukuphepha iingxaki zempilo yomntwana.

Ukuze bazikhusele kwisifo seCreutzfeldt-Jakob, kucetyiswa ukuba inyama akufanele idliwe kwimimandla apho iziganeko zezifo zeenkomo zibhalwe. Okokuqala, sithetha ngamazwe aseYurophu. Kwakhona, musa ukusebenzisa unyango lweziyobisi ezenziwe kwigazi lezilwanyana okanye abantu. Kukulungele ukutshintsha indawo yazo ngeendlela ezifanayo.

Izifo ze-Prion zifundwa ngokungafunekiyo iifom zezifo ezithathelwanayo kunye nezifa ezivela emzimbeni womntu malunga nemvelaphi yokungena kweeprotheni ezingavamile. Kwiimeko ezininzi, zichaphazela inkqubo yesantya. Umfanekiso weklinikhi ubonakaliswa yimpawu ezifanayo. Ekuqaleni, ukutya komntu kunye nombono ulahlekile, ukulungelelaniswa kwendawo kuphazamiseka. Kwinqanaba lokugqibela, ukugula komzimba kukhula, xa isiguli singenakuzinyamekela. Isiphumo saso nasiphi na isifo soloko sifanayo - ukufa. Okwangoku, oogqirha abanalo iindlela ezifanelekileyo zokuchasene nezifo zolu hlobo.