Prion zvirwere: zvinokonzera, zviratidzo, nechirwere, kurapwa

zvirwere Prion - iri chakakomba neurodegenerative zvirwere vanhu nemhuka chinokosha. Ivo vanoonekwa ne mberi uropi, kakawanda kupera kufa nokukurumidza.

Ndezvipi prions?

Ichi chinokosha mapuroteni mamiriro. Vanogona kuva zvose zvinoitika uye chikamu munyama vanhu utano uye mhuka, uyewo kanganisika, zvichiita-siyana zvirwere. Kusvikira Mumakumi emakore akapfuura, yakanga vaidavira kuti vapenyu mamiriro vanofanira zvine dzinonzi nucleic acids - RNA uye DNA. Pamusana kwavari, zvinokwanisika zvichiberekana. Nemavhairasi, notumwe tupukanana shiri, mhuka "vanoumba" nucleic acids. Kare zvakanzi vaifungidzira kuti kusavapo kwavo nyama Hazvibviri zvakazara bereka. Prion mapuroteni anoposhwa pfungwa idzi zvachose.

mamorekuru aya akaumbwa chete mapuroteni, asi zviri kusiyaniswa kukwanisa bereka. Rakapinza muruoko muviri, vasingagumbusi kushandurwa zviri mukati mune kuipa pathological prions, nokudaro vachiwedzera nhamba yavo. Izvi zvinoda nguva yakawanda kupfuura kubereka mabhakitiriya kana mavhairasi, saka kubvira panguva vachipinda muviri Morekuru kuti kukura chirwere anogona kutora makore akawanda.

The ehupfumi kuti prions

Prions uye nezvirwere prion vari dzinoratidzwa yakakwirira nemishonga. Nzira dzakawanda disinfection hadzibatsiri mukubata navo. Prions hadzina kuparadzwa kubika, angakudzivisai kutonhora kusvika -40 Celsius. Havadi kuratidza aikanganiswa UV nemwaranzi uye kuramba zvivako zvavo panguva processing formalin.

Zvakakurukurwa sechivako mapuroteni mamorekuru kunotungamirira kuti muviri haagoni kurwa navo. Haasi inokwanisa kubudisa antibodies pamusoro prions, kwete kushora lymphocytes avo sevasingaoni. Izvi zvinoreva kuti kupinda pakati mamorekuru mumuviri womunhu rinobatanidza panowanikwa chirwere.

Prion zvirwere: nhoroondo yakawanikwa

Muna 1982, Stenli Pruziner kutanga akarondedzera prion zvirwere, yaakanga pashure paakavapa Nobel Prize.

Kare kare yawanika, masayendisiti mubasa ravo akaongorora anoverengeka pathologies dzevanhu uye mhuka, iyo aisagona kutsunga kwenguva refu. Muzana XVIII remakore muUnited Kingdom rakachengetedzwa vakanyorwa, "scrapie" makwai. Animals chirwere chakakomba kuvaviwa, kufamba zvirwere uye nezvipusha, izvo akataura kuna CNS. Muna 1957, Daniel Carleton Gajdusek akarondedzera zvirwere Pamberi rudzi, vane vanhu vaigara yemugomo kuPapua New Guinea. The zvokurwara aishamwaridzana kudya vanhu uye vakapfuura pamunhu nomunhu mumwe.

Kubvira muna 1986, muEngland, uye gare gare mune dzimwe nyika dzakawanda, masayendisiti akanyora panomuka shoma vakazopiwa zita "kupenga mhou chirwere". It runonyanya inokanganisa mombe. "Mad mhou chirwere" pashure pfupi nguva yasvika denda zvikuru, uye chikonzero parinowanika vava prions. Mumakore 90 vanamazvikokota nekusimba kupararira chirwere ichi kumunhu pamwe mukaka nenyama mombe.

Pari, chidzidzo hwakadzama pamwe undiagnosed zvinokonzera zvirwere chave chokwadi kuti masayendisiti akaita nhamba vaitaura kuti kukura prion hunhu. Pakati pavo pane zvokurwara pamusoro Creutzfeldt-Jakob chirwere, chirwere cheAlzheimer. Zviratidzo nezviratidzo zvirwere izvi vane zvakawanda zvakafanana. Pasinei hombe mberi mukudzidza zvinetso izvi, zvikuru inogara zvinopfuura inonzwisisika.

Unopinda sei utachiwana?

In mushonga ano, pane nhatu nzira chirwere.

1. Transmissible. Prions vari achichinjwa mammalian mhando kune mumwe. Yambotaurwa ariko anonzi marudzi nokuidzivirira. Izvi zvinoreva kuti kuchinjwa kubva mhou hazvibviri murume. Masayendisiti ndeyenhema maonero ichi. Protein mamorekuru hunogona kutapukira kune utachiona mhuka kana munhu. Zvinokonzera nezvirwere prion anokonzerwa ingestion yenyama / neutachiona mhuka mukaka, kushandiswa zvipenyu rinenge (corneal transplantation, zvinogadzirwa neropa, uye zvichingodaro. D.). Various biomaterials inosiyana siyana pamusoro pathogenicity. Mukuru infectivity ratidza uropi, danho rinotevera inogarwa ropa uye ropa zvigadzirwa.
2. Nhaka. Hosha ikabuda pamberi nemagene kunotouraya, zvichiitika rechi20 chromosome. Hazvina nzvimbo iri kukonzera kuvapo inowanzotorwa prion mapuroteni. mashandiro aro achiri havaratidzi kuzivikanwa. Kana Gene kunotouraya panzvimbo prion utano pathological synthesized, izvo vanotozvikuvadza inotungamirira kuvapo kwezvirwere.
3. Nguva dziri kure (vaingoerekana panowanikwa yakaoma mapuroteni).

Saka, zvirwere prion kunogona nhaka uye rwarisa hunhu. Pasinei nzira kupinda pakati zvemamiriro mapuroteni pamuviri, zvinogona kukonzera chirwere vamwe.

Chii chinokonzera prions mumuviri?

Kupera mapuroteni dzinoratidzwa kwavo kukwanisa kukonzera spongiform encephalopathies, kureva CNS. Kubva morphological nemaonero, ichi chinoreva nokuumbwa in muuropi mumasero, neuronal rufu, overgrowth panzvimbo yavo uye kugumisa connective zvinyama atrophy wouropi. Tikafunga paumbwe masumbu prion amyloid plaques kuchengetwa. pezvakaitwa zvose izvi zvichiitika pasina zviratidzo pachena kuzvimba.

Ndezvipi zvirwere zviri prion?

To musi, masayendisiti anogona zvakarurama kungotaura zvirwere shoma, inova mhaka zvemamiriro mapuroteni zvivako:

• Creutzfeldt-Jakob chirwere;
• kuru chirwere;
• Alpers (chirambe chakapfava mberi encephalopathy) chirwere;
• zvinouraya rwapedyo kushaya hope;
• chirwere Gerstmann-Shtreusslera-Scheinker.

Next, tarisa nomumwe muudzame yacho zvokurwara.

Creutzfeldt-Jakob chirwere

Creutzfeldt-Jakob chirwere kunoratidzwa ayo siyana, saka nyanzvi razdeleli muna dzakawanda:

• nguva dziri kure;
• mhuri;
• iatrogenic;
• itsva atypical chimiro.

Nguva dziri kure musimboti chirwere kare vaiona kupfuura zvakapararira. Zviratidzo ake okutanga pashure pemakore 55. Zvisinei, mumakore mashoma apfuura makore nhamba zvachinja. Pashure nechitarisiko mashoko pamusoro kupararira "kupenga mhou chirwere" vari kuramba akatanga kunyora nyaya atypical siyana nokuda mombe hweHIV. Nekuti hunhu yapfuura nechitarisiko mhando iyi. Kazhinji, vechidiki vanotambura. Zviratidzo zviri mapoka mapoka maviri zvimiso: chetsinga uye vairwara. Pakutanga, chirwere nemuumbi nemusoro, hope chinovanetsa, ungaderedza kudya. Zvishoma nezvishoma, Zviratidzo izvi akatizve kuyeuka vanotaura, kurasikirwa wezvakaonekwa. Chepfungwa zvinozarurwa muchimiro zvisipo uye zvinovadadira. The chirwere kunoratidzwa kufambira kukura mune rokupedzisira Danho kunoratidzwa kwakakwana kutadza kufamba muviri. Munhu anotsamwa pamusoro kushanda pelvic zvomukati. With chirwere chakadai vanhu vanorarama kupfuura makore maviri.

The pakuonekwa mhuri chimiro kunokonzerwa kunotouraya ari Gene pamwero ari mapazi ari rechi20 chromosome. Maperembudzi ichazivikanwa noku autosomal kureva hunhu. Zviratidzo zvokutanga kuitika anenge 5 makore adarika kupfuura nguva dziri kure chimiro.

Iatrogenic chimiro kukura somugumisiro chirwere hwevanhu pakuvhiya. Statistical mashoko nezvechisarudzo ichi hachisi chirwere, sezvo zvakaoma kuratidza pathogenesis zvirwere prion. The incubation nguva anoda kubva 7 months makore 12. Zvinonzi kunoyerwa ibatanidzo zvinoverengeka: chirongwa kupinda pakati kanganisika mapuroteni pamuviri, nokuwanda, pakutanga genotype munhu. The kukurumidza kuwedzera chirwere kubudikidza zvakananga kupinda nokuda prions mu uropi somugumisiro kuvhiyiwa. More nguva kunodiwa panguva chirwere pamusoro pakanyorwa corneal transplantation kana reDhura mai. Varwere zvishoma nezvishoma kuva cerebellar ataxia, havachanyatsogoni matauriro uye tsandanyama matauriro, dementia.

"Mad mhou chirwere" akatanga kuwana kushanda pashure denda iri kuzvipfuwo 90s. Prion chirwere, zviratidzo zvinoonekera pakati pemakore 30 kusvika 40 makore, ndiyo inouraya vanhu. Sezvazviri iatrogenic chimiro, chetsinga zviratidzo vakawanda pamusoro mupfungwa.

Kanyanyisa rwapedyo kushaya hope

chirwere ichi ane autosomal hunonyanya hunhu, icho chinopfuudzwa chete yakatevedzana. Zvinouraya kushaya hope hariwanikwi. Iye zvinozivikanwa sayenzi kubva 1986. Zviratidzo Its yokutanga pakati makore 25 kusvika munenge 71 mugore.

Epidemiology pamusoro prion zvirwere yakadaro insufficiently Akaongorora. The chikuru chiratidzo dzinouraya rwapedyo kushaya hope ndiyo atadze kurara. The muviri zvishoma nezvishoma anorasikirwa kukwanisa rayo zvakazara kuchinja chehupenyu takamuka uye hope. Uyewo, varwere kuoneka kufamba zvinetso uye tsandanyama simba. Kwave zviitiko autonomic zvirwere kuti pachena uye tinoratidza pachena zvakawedzera BP, kunyanya vangadikitira. Of chirwere chepfungwa zvinogona kucherechedzwa vhunduka, ziso zvisipo, uye inoratidza Zvikamu kuvhiringika. Nekuti hope kugara kuneta kunoitika, murwere anofa.

Kuru

Infectious prions chimiro vakanga vadzidza zvakadzama nokuda chirwere, zvikuru zvakananga, rudzi cannibals. Kusvikira muna 1956 pakati vagari Papua - New Guinea akaparadzirwa vanonzi tsika tsika kudya vanhu - vachidya uropi emunhu akafa. Zvinofungidzirwa kuti rimwe nhengo rudzi urwu vakasimuka utachiona, gare gare kupararira kune dzimwe vanhu pashure tsika. Sezvo Kudzimwa tsika iyi zvirwere zviitiko yakanyorwa kakawanda zvishoma kazhinji nhasi chirwere ichi kushanda zvikasaitika.

The incubation nguva anoda kubva 5 kusvika 30 makore. Ndokusaka Kuru chirwere rinotaurwa se "anononoka utachiona hweHIV" muchikwata. Chirwere cerebellar zvirwere hwakaratidzwa pamwe chin'a kuseka, pakuparadza dysfunction uye tsandanyama simba. In chinouraya nzendo dzichiri dementia. Vanhu vane chirwere ichi kurarama asina kupfuura mwedzi 30.

Alpers chirwere

Hosha kunyanya rinowanikwa mune vana vaduku (pasi pemakore 18). Kurwara iri chinopfuudzwa ari autosomal recessive nenzira, panyaya masanga vaviri pathogenic majini vanababa navanaamai. Pakati huru zviratidzo vangazivikanwa madzerere uye pfari. In zvokurapa mabhuku pane kurondedzerwa rikwanise chirwere yokuvamba nokuda nesitiroko mhando. Alpers chirwere chiri dzinoratidzwa maronda chiropa, iyo inokurumidza achikura kuva chisingaperi kuzvimba chiropa uye anopera cirrhosis. Varwere vari kufa pamusana kudhaka kwemwedzi 12 mushure chirwere yokutanga zviratidzo.

Gerstmann chirwere-Shtreusslera-Scheinker

Ipfungwa Izvi semunhu nhaka chirwere mhando. Very kushoma (imwe nyaya vanhu vanenge mamiriyoni 10). Zviratidzo zvokutanga anowanzoshandurwa kuonekwa varwere kwemakore 40. Kukura chirwere kunotanga cerebellar zvirwere. Pakutanga dzungu anooneka. Sezvo chirwere mberi koordinatornye kutyorwa zvishoma nezvishoma akazvimirira kufamba zvisisakwanisiki. Pamwe chete vakanyorwa zviratidzo kuonekwa kusagadzikana tsandanyama matauriro, vasanyanye muchiratidzo uye kunzwa, nezvinetso kumedza uye kubereka. In magumo-Danho pazvinhu vanachiremba kuratidzirwa dementia. Hunotarisirwa upenyu varwere ichi chirwere kusvika makore 10.

CheAlzheimer uye Parkinson chirwere

CheAlzheimer uye Parkinson, chirwere zviratidzo nezviratidzo zvacho zvakafanana hunhu, kukudziridza nenzira yakafanana kuti prion zvirwere. Mamorekuru Akaoma Beta amyloid, rokuti tau mapuroteni nedzimwe nhengo uyewo kuumba deposits ose pathogenic zviriko uropi. Zvisinei, kubata zvirwere izvi hazviiti. Izvi zvinoreva kuti amyloid fibrils vari inoumbwa tagovera mapuroteni mamorekuru, asi "utano" chiito "kurwara" haushandi.

Munguva ichangopfuura, masayendisiti ichiitwa nhevedzano zvidzidzo zviri mbeva, izvo akaramba kubvuma ichi. Pashure pakatanga pathogenic mapuroteni muuropi iri zvachose mhuka utano ane maitiro amyloid plaques. Izvi zvinoreva kuti nayo utachiona mapuroteni vanongogona kutapura chivakwa utano. The Kuwanika ndohwaJehovha vanachiremba kubva University of Texas. Munguva yemberi iri pedyo achauya rimwe basa masayendisiti kubva London, izvo zvinoratidza kuti cheAlzheimer, zviratidzo uye zviratidzo chirwere chinogona kuva vakapfuura kubva kune mumwe munhu.

Yeuka kuti chirwere chinonzi Parkinson kunoratidzwa mberi kurasikirwa Tutsinga tunofambisa mashoko utwu kuti vabereke dopamine, mumwe neurotransmitter. Nokuda kwaizvozvi, munhu akashatirwa murau nemafambiro uye tsandanyama tonus, uko kunoratidzwa tremor, kunopindirana kuomarara. Parkinsonism anotambura ose ramazana munhu akatendeuka matanhatu kunze. The chirwere anotanga kukura waro anonoke inofamba, izvo kana munhu akapfeka kana zvinotora kudya kunonyanya kuoneka. Zvadaro, kuputswa kutaura, pakuparadza hunenge. Zvinosuruvarisa kuti mushonga arege kutokurudzira zvinobudirira kurapa vanhu vane chirwere che "chirwere chinonzi Parkinson". Zviratidzo uye zviratidzo chirwere ichi chinogona mitigated nokuda symptomatic kurapa. Zvisinei, dzakawanda zvinodhaka izvi zvinokonzera zvinoverengeka nemishonga.

CheAlzheimer - chirwere inozivikanwa nokuda unooma kuti Tutsinga tunofambisa mashoko utwu, zvichiita varwere kuva dementia. Zviratidzo chirwere ichi pakutanga anogona kuitika kare zera 40. Zvinonzi chirwere kakawanda ruzivo vanhu. Munhu mukuru pamwero yenjere mukana nani kutsungirira zviratidzo yeAlzheimer nokuda zhinji kubatana pakati Tutsinga tunofambisa mashoko utwu.

The chirwere anotanga kukura kwayo nendangariro zvirwere. The chikuru Danho Kazhinji anoenda rwakaonekwa nevamwe. The kutanga Zviratidzo kazhinji kuedza kuvhara kana mhosva mwoyo uye kunyanya pokuwanda kwebasa kubasa. Sezvo kuchinja ari kurapwa mufananidzo chirwere mutates. Murwere hakuchisina dzokumabvazuva pachavo vari muchadenga, kubva ake ndangariro inodonha kare vawana unyanzvi kunyora, kuverenga. Chokutanga, kukanganwa inotevera chiitiko nenguva. Kana zvokurwara anotanga mberi, zvinokosha kushandisa mukana wose kuchengetedza munhu anogona kuzvicheka-basa, kuedza kudzivirira panowanikwa mwoyo. Kugadzirisa Dambudziko iri rinogona kubatsira zvikuru simba kunzwa rubatsiro kana zvakakodzera yakanyatsoiswa magirazi. Dzakananga Mabatirwo yeAlzheimer chirwere haako. Kana chikuru zviratidzo zvakakosha kuwana kuongorora wakakwana pedyo wetsinga. nokuti mishonga nyanzvi kazhinji dzinokurudzira zvapanzvimbo chirwere kuti zviite uye kumumanikidza kukura kwayo.

Chirwere zvirwere prion

Dzinenge matanho chirwere havasi parizvino kumiririrwa. Somuenzaniso, kufanana eeg Zvakawanikwa sezvaakaita Creutzfeldt-Jakob chirwere, vari dzimwe pfungwa pathologies. It dzinoratidzwa pasi MRI chirwere kukosha, kubva 80% of varwere zvinoratidza non-zvakananga anonongedzera. Zvisinei, kudzidza ichi anotibvumira kuziva pfungwa atrophy. nekuomarara Its uri kuwedzerwa sezvo mberi zvirwere zvevanhu prion.

The differential chirwere rinoitwa vose pathologies, mumwe kuratidzwa inova dementia (cheAlzheimer, vasculitis, neurosyphilis, herpes encephalitis nevamwe).

Treatment Nzira

Zvinosuruvarisa iye, zvirwere zvose prion vari chisingarapiki. Varwere basa symptomatic kurapa pamwe anticonvulsants, izvo chete zvinoderedza kutambura. The kushurudzira mwoyo. anozivikanwa zvirwere zvose prion zvinouraya hwevanhu.

Pari, masayendisiti munyika yose vari kushingaira kutsvaka mushonga zvakasikwa. Tsvakurudzo kushandisa mhuka. Zvinofungirwa kuti mukurwisa zvirwere hunde masero dzakadaro ichashandiswa gare gare, uyewo inonyanya mbiriso. zvinodhaka experimental Pari havana yakakwirira kunyatsoshanda, saka chinangwa chavo anonzi kukodzera.

matanho

Kubva pave nguva dziri kure uye nhaka dzakasiyana zvirwere prion kuzvidzivirira kutonetsa. Zvimwe zvirwere zvinogona kubviswa nokuisa nemagene kuongorora chaiyo. Zvisinei, zviri munyika yedu zvakaoma chaizvo, nokuti murabhoritari kuita kuti nechirwere mhando ichi, vari zvikurukuru kunze.

Kana nhaka nezvirwere vasati pamuviri vari zano kubvunza chiremba-geneticist. Izvi zvichakubatsira kudzivisa matambudziko hwamangwana hutano mwana.

Kuti vazvidzivirire kubva Creutzfeldt-Jakob chirwere, Zvinokurudzirwa kuti kurega kudya nyama kubva munzvimbo zviitiko mombe chirwere akanyorwa. isu Chekutanga vari kutaura nyika dzokuEurope. Unofanira kwete kushandiswa pakurapa kugadzirira wakaita neropa remhuka kana vanhu. Zviri nani kuti todzitsiva chakaita zvokugadzirwa analogues.

Prion zvirwere - haasi zvakakwana kudzidza siyana zvakawanda zvinotapukira uye enhaka maronda kuti kuitika mumuviri womunhu ari pakanyorwa kupinda pakati mwero mapurotini. Kazhinji, ivo zvazvinoita musimboti tsinga. The mufananidzo zvekuchipatara kunoratidzwa zvakafanana zviratidzo. Pakutanga, mumwe munhu akarasikirwa kudya uye zvakaratidzwa, vavhunduke Kufambisa muchadenga. Panguva yokupedzisira padariro dzichiri dementia apo murwere haakwanisi vakazvimirira ndinovachengeta pachangu. Mugumisiro chero chirwere nguva dzose chete - rufu. Pari, vanachiremba havana zvinobudirira mushonga pamusoro pathologies zvakasikwa ichi.