Yini iJAK2 Gene?

-Delile

• Ukubuka konke
• Izakhi zofuzo nokugula
• Umsebenzi ojwayelekile we-JAK2
• I-JAK2 nezifo zegazi
• Ucwaningo lwe-JAK2

Ukubuka konke

I-enzyme ye-JAK2 bekuyiyona nto obekugxilwe kuyo ocwaningweni muva nje ekwelashweni kwe-myelofibrosis (MF). Enye yezindlela zokwelashwa ezintsha nezithembisa kakhulu ze-MF umuthi omisa noma wehlise ijubane ukuthi i-enzyme ye-JAK2 isebenza kangakanani. Lokhu kusiza ukunciphisa isifo.

Qhubeka ufunde ukuze ufunde nge-enzyme ye-JAK2, nokuthi ihlobana kanjani nofuzo lwe-JAK2.

Izakhi zofuzo nokugula

Ukuqonda kangcono isakhi sofuzo ne-enzyme ye-JAK2, kuyasiza ukuba nokuqonda okuyisisekelo kokuthi izakhi zofuzo nama-enzyme asebenzisana kanjani emizimbeni yethu.

Izakhi zethu zofuzo ziyiziyalo noma amapulani wemizimba yethu ukuze asebenze. Sinesethi yale miyalo ngaphakathi kwawo wonke amaseli omzimba wethu. Batshela amangqamuzana ethu ukuthi awenza kanjani amaprotheni, aqhubeka nokwenza ama-enzyme.

Ama-enzyme namaprotheni athumela imiyalezo kwezinye izingxenye zomzimba ukwenza imisebenzi ethile, efana nokusiza ngokugaya ukudla, ukukhuthaza ukukhula kwamaseli, noma ukuvikela umzimba wethu ezifweni.

Njengoba amangqamuzana ethu ekhula futhi ehlukana, izakhi zofuzo ezingaphakathi kwamaseli zingathola ukuguquka komzimba. Iseli lidlulisela lokho kuguquka kuwo wonke amaseli eliwakhayo. Lapho isakhi sithola ushintsho, kungenza amapulani kube nzima ukuwafunda.

Kwesinye isikhathi, ukuguquka kwesimo kudala iphutha elingafundeki kangangokuba iseli alikwazi ukudala amaprotheni. Kwesinye isikhathi, ukuguqulwa kuguqula iprotheni ukuthi kusebenze isikhathi esengeziwe noma ukuhlala ivuliwe njalo. Lapho ukuguquka kokuphazamisa ukusebenza kwamaprotheni ne-enzyme, kungadala izifo emzimbeni.

Umsebenzi ojwayelekile we-JAK2

Uhlobo lwe-JAK2 lunikeza amangqamuzana ethu iziyalezo zokwenza iphrotheni ye-JAK2, ekhuthaza ukukhula kwamaseli. Uhlobo lwe-JAK2 ne-enzyme kubaluleke kakhulu ekulawuleni ukukhula nokukhiqizwa kwamaseli.

Babaluleke ngokukhethekile ekukhuleni nasekukhiqizeni amangqamuzana egazi. I-enzyme ye-JAK2 isebenza kanzima kumaseli esiqu emnkantsheni wethu wethambo. Eyaziwa nangokuthi ama-hematopoietic stem cells, lawa maseli anesibopho sokwakha amangqamuzana egazi amasha.

I-JAK2 nezifo zegazi

Izinguquko ezitholwe kubantu abane-MF zidala ukuthi i-enzyme ye-JAK2 ihlale ivuliwe. Lokhu kusho ukuthi i-enzyme ye-JAK2 isebenza njalo, okuholela ekukhiqizweni ngokweqile kwamaseli abizwa ngama-megakaryocyte.

La ma-megakaryocyte atshela amanye amaseli ukuthi akhiphe i-collagen. Ngenxa yalokhu, izicubu ezibomvu ziqala ukwakha emnkantsheni wethambo - uphawu lwe-telltale lwe-MF.

Ukuguquka kwezakhi zofuzo ku-JAK2 kuxhumene nokunye ukuphazamiseka kwegazi. Okuvame kakhulu, ukuguqulwa kuguqukele esimweni esibizwa nge-polycythemia vera (PV). Ku-PV, ukuguqulwa kwe-JAK2 kubanga ukukhiqizwa kwamangqamuzana egazi okungalawulwa.

Cishe amaphesenti ayi-10 kuye kwayi-15 abantu abane-PV bazoqhubeka nokuthuthukisa i-MF. Abaphenyi abazi ukuthi yini ebangela abanye abantu abanezinguquko ku-JAK2 ukuthi bathuthukise i-MF kanti abanye bakhulisa i-PV esikhundleni.

Ucwaningo lwe-JAK2

Ngoba izinguquko ze-JAK2 zitholakale kubantu abangaphezu kwengxenye yabantu abane-MF, nangaphezulu kwamaphesenti angama-90 abantu abane-PV, kube yisihloko semiklamo eminingi yocwaningo.

Kunomuthi owodwa kuphela ogunyazwe yi-FDA, obizwa nge-ruxolitinib (Jakafi), osebenza nama-enzyme e-JAK2. Lesi sidakamizwa sisebenza njenge-JAK inhibitor, okusho ukuthi kubambezela umsebenzi we-JAK2.

Lapho umsebenzi we-enzyme wehliswa, i-enzyme ayivulwa ngaso sonke isikhathi. Lokhu kuholela ekukhiqizeni okungaphansi kwe-megakaryocyte kanye ne-collagen, ekugcineni kunciphise ukwakheka kwezicubu ezibomvu ku-MF.

Isidakamizwa i-ruxolitinib futhi silawula ukukhiqizwa kwamaseli egazi. Lokhu ikwenza ngokunciphisa umsebenzi we-JAK2 kumaseli ezinhlayiya ze-hematopoietic. Lokhu kwenza kube lusizo kuzo zombili i-PV ne-MF.

Njengamanje, kunezivivinyo eziningi zomtholampilo ezigxile kwamanye ama-JAK inhibitors. Abaphenyi basebenza nokuthi bangasisebenzisa kanjani lesi sakhi sofuzo kanye ne-enzyme ngethemba lokuthi bazothola ukwelashwa okungcono noma ikhambi le-MF.