Chirwere cheCreutzfeldt-Jakob chimwe chezvirwere zveprion. Izvi zvirwere zvisingawanzo zivikanwa nekudzikira kwepakati tsinga system uye zvinonziwo subacute transmissible spongiform encephalopathies (TSE). Izvo zvinokonzerwa nekuunganidza muuropi yeprotein yakajairika asi isina kurongeka, iyo prion protein (1). Nehurombo, chirwere cheCreutzfeldt-Jakob chinoratidzwa nekukurumidza uye inouraya nzira pamwe nekusavapo kwekurapa. Kune zana kusvika zana nemakumi mashanu emakesi gore rega rega muFrance (100).

Chirwere ichi kazhinji chinotanga nekusarongeka kusingatarisirwi sekushaya hope kana kufunganya. Zvishoma nezvishoma, kuyeuka, kutaridzika uye kukanganisa kwemutauro kunoiswa mukati. Inobva yaratidzwa nezvirwere zvepfungwa pamwe necerebellar ataxia (kusagadzikana kana wakamira usingafambi uye panguva yekufamba iyo inoperekedzwa nekudengenyeka kwakafanana nekudhakwa). Kune zvakare maronda akajairwa mukati mepakati tsinga system (florid plaques, amyloid deposits yePrPres yakakomberedzwa nevacuoles).

Vose varume nevakadzi vanobatwa, zvisinei nehuwandu hwepamusoro muvakuru vechidiki.

Sezvineiwo, hapana yakavimbika diagnostic test. Electroencephalogram (EEG) inogona kuona kuvhiringika kwakanyanya mukuita kwehuropi. MRI inotaridza kusarongeka chaiko mune mamwe matunhu ehuropi (basal ganglia, cortex) uko kune kushoma kwakasiyana kuongororwa.

Kana zvese izvi zvekiriniki uye paraclinical zvinhu zvinogona kuita kuti zvikwanise kuita kuongororwa kwechirwere cheCreutzfeldt-Jakob, ingori fungidziro inogoneka: kutaura zvazviri, kuongororwa chete kwehuropi tishu, kunoitwa kakawanda mushure mekufa kunobvumira kusimbisa kuongororwa.

Chirwere cheCreutzfeld-Jakob ndicho chega chirwere chevanhu chinogona kunge chiri chikonzero chemajini (nekuda kwekushanduka kwejini encoding iyo prion protein, iyo E200K mutation ndiyo inonyanya kuzivikanwa), inotapukira chikonzero (chechipiri kune kusvibiswa) kana che sporadic fomu (ye kuitika zvisina tsarukano, pasina shanduko kana kuratidzwa kune exogenous prion inowanikwa).

Nekudaro, iyo sporadic fomu ndiyo inonyanya kuzivikanwa: inoverengera makumi masere neshanu muzana eese subacute transmissible spongiform encephalopathies (TSEs) anoongororwa gore rega rega. Muchiitiko ichi, chirwere chinowanzoonekwa mushure memakore makumi matanhatu uye chinopfuurira mukati memwedzi inenge mitanhatu. Kana chirwere chacho chiri chemajini kana utachiona, zviratidzo zvinotanga uye zvinofambira mberi zvishoma nezvishoma. Mune hutachiona hwehutachiona, nguva yekunyungudika inogona kureba zvakanyanya uye kudarika makore makumi mashanu.

Iyo prion protein (PrPc) ipuroteni yepanyama inowanikwa nenzira yakanyatsochengetedzwa mumarudzi akawanda. Muuropi neurons, puroteni yeprion inogona kuve pathogenic nekushandura yayo-matatu-dimensional conformation: inopeta pairi pachezvayo zvakasimba, izvo zvinoita kuti ive hydrophobic, inonyungudika zvishoma uye inoshingirira kuderera. Inobva yanzi "scrapie" prion protein (PrPsc). PrPsc inoungana pamwe chete uye inogadzira madhipoziti anowanda mukati nekunze kwemasero ehuropi, zvichikanganisa basa ravo uye nzira dzekupona.

Mune iyi fomu isina kujairika, puroteni yeprion inokwanisawo kuendesa kusarudzika kwayo: pakubata nePrPsc, puroteni yakajairwa yeprion inotora chimiro chisina kujairika. Iyi ndiyo domino effect.

Ngozi yekutapurirana pakati pevanhu

Kutapurirana kwezvirwere zveprion kunogoneka nekutapurirana tishu kana kutevera kutungamirwa kwekukura kwehomoni. Matishu ane ngozi zvakanyanya anobva pakati penheyo system uye ziso. Kune zvishoma, cerebrospinal fluid, ropa uye dzimwe nhengo (itsvo, mapapu, nezvimwewo) zvinogonawo kutumira prion isina kujairika.

Ngozi yechikafu

 Kutapurirwa kweprion kubva kumombe kuenda kuvanhu kuburikidza nekudyiwa kwechikafu chakasvibiswa kwakafungidzirwa muna 1996, panguva yedambudziko rakakura re "mombe inopenga". Kwemakore akati wandei ikozvino, denda rebovine spongiform encephalopathy (BSE) rarova mapoka muUnited Kingdom3. Kupararira kwechirwere ichi cheprion, icho chakabata makumi ezviuru zvemhuka gore rega rega, pasina mubvunzo chaive nekuda kwekushandiswa kwehupfu hwemhuka, hwakagadzirwa kubva muzvitunha uye husina kukwana hwakasvibiswa. Zvisinei, kwakabva kwaita nharo.

Nhasi, hapana kurapwa kwakananga kwezvirwere zveprion. Mishonga chete inogona kurongerwa ndeye iyo inogona kusunungura kana kuderedza zviratidzo zvakasiyana-siyana zvechirwere. Rutsigiro rwekurapa, rwemagariro uye rwepfungwa rwunopihwa kuvarwere nemhuri dzavo neNational CJD Support Unit. Kutsvaga kwemishonga ine chinangwa chekudzivirira kutendeuka kwePrPc, kukurudzira kubviswa kwemaitiro asina kujairika eprotein uye kuderedza kupararira kwayo kune tariro. Inonakidza inotungamira inonongedza PDK1, imwe yevamiriri venhare vanobatanidzwa panguva yehutachiona. Kudzivirirwa kwayo kwaizoita kuti zvikwanisike zvose kuvharidzira chiitiko chekushandura kuburikidza nekusimudzira cleavage yePrPc, uye kuderedza migumisiro yekudzokorora kwayo pakupona kwema neuroni.