4. December 2024

Genforskning på sebrafisk avslørte alvorlig sykdom som kun rammer menn. Genet er lokalisert i X-kromosomet

 

https://www.nettavisen.no/nyheter/genforskning-pa-sebrafisk-avslorte-alvorlig-sykdom-som-kun-rammer-menn/s/12-95-3424182705

  • Genforskning på sebrafisk avslørte alvorlig sykdom som kun rammer menn. – Vi har ingen helbredende behandling, sier legen som så langt har påvist sykdommen hos fem norske menn.

I GENENE: Genet som forstyrres er lokalisert i X-kromosonet, som menn kun har ett av. Dermed er de også mer sårbare for denne nyoppdagede sykdommen. Illustrasjonsbilde viser undersøkelse av DNA-fragmenter i et laboratorium.
I GENENE: Genet som forstyrres er lokalisert i X-kromosonet, som menn kun har ett av. Dermed er de også mer sårbare for denne nyoppdagede sykdommen. Illustrasjonsbilde viser undersøkelse av DNA-fragmenter i et laboratorium. Foto: Wong Maye-e (AP)

– Vi har ingen helbredende behandling, sier legen som så langt har påvist sykdommen hos fem norske menn.

Den alvorlige sykdommen er kun påvist hos menn og skyldes mutasjoner i et gen som koder viktige biologiske prosesser. Hvorfor det skjer, vet legene ikke. Noen helbredende behandling har de heller ikke, og sykdommen kan være dødelig.

Sykdommen har fått navnet VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic). Syndromet omtales på norsk som «alvorlig inflammatoriske sykdom hos voksne menn».

– Vi hørte om dette første gang i november 2020, og vi har så langt påvist sykdommen hos fem pasienter på Oslo universitetssykehus (OUS), sier Øyvind Midtvedt, overlege ved revmatologisk seksjon, til Nettavisen.

Den første pasienten gikk bort, og det var først etter hans død at legene koblet hans sykdomstilstand til det nyoppdagede syndromet.

– Vi forstod ut fra det vi hadde lest i fagtidsskrifter at dette kunne være VEXAS-syndromet. Vi tok kontakt med ektefellen til avdøde og fikk tillatelse til å gjøre genetiske tester. Dette er en sykdom som kun kan bekreftes gjennom genetisk analyse, en analyse som er tilgjengelige i Norge. For legene som behandlet pasienten gjennom mange år og ikke minst for de pårørende, var det en lettelse at en endelig kunne stille riktig diagnose, forklarer han.

Øyvind Midtvedt, overlege ved revmatologisk seksjon ved Oslo universitetssykehus.
Øyvind Midtvedt, overlege ved revmatologisk seksjon ved Oslo universitetssykehus. Foto: Privat

Rammer middelaldrende menn

Det synes å være middelaldrende menn som er utsatt. Sykdommen gir symptomer som nedsatt allmenntilstand, feberepisoder, betennelse i nese- eller ørebrusk (kondritt), de kan ha utslett og de kan ha forandringer i lunger. Lav blodprosent, blodpropp, høy blodsenkning og høy CPR som tegn på kraftig betennelse er også kjennetegn.

– Sykdommen er ikke medfødt, og den er ikke smittsom. Vi kjenner ikke risikofaktorene. Syndromet er sjeldent, men sannsynligvis underdiagnostisert på grunn av manglende kjennskap til tilstanden.

Vi behandler symptomene med kortison og betennelsesdempende medisiner, sier Midtvedt og sier norske leger har hatt dialog med utenlands ekspertise på området.

– Siden det dreier seg om endringer i et gen, har vi ingen helbredende behandling. Så langt har vi ikke kommet, men utviklingen med raskere og billigere gensekvensering er til hjelp for diagnostikk av sykdommen, sier han.

Forsket på sebrafisk

Midtvedt forteller at de amerikanske forskerne som først påviste VEXAS-syndromet begynte med å se på et pasientregister med uklare betennelsessykdommer. Så fant de en fellesnevner, en mutasjon, det vil si en endring i et gen (UBA1-gen). Genavviket forårsaker en forstyrrelse i en sentral biologisk prosess som igjen fører til kraftig betennelse. I et forsøk der de genmanipulerte sebrafisk klarte de å framkalle sykdom hos fisk.

Dermed kunne de gi en beskrivelse av sykdommens symptomer og si at den var forårsaket av mutasjon i det aktuelle genet. Genet er lokalisert i X-kromosomet, som menn kun har ett av. Det er grunnen til at menn er mer sårbare for denne sykdommen.

– Det at vi ser at endringer i et gen gir slike betennelsesaktige syndromer er interessante funn og bør åpne opp for mer forskning, sier Midtvedt til Nettavisen.

Også påvist i Danmark

To leger ved Rigshospitalet i Danmark har nylig gått ut og fortalt at de har kunnet påvise sykdommen hos fem pasienter av en gruppe på 1000 med hittil uforklarlige symptomer.

 

De danske legene fant de samme genetiske mutasjonene, hematologisk og revmatologiske symptomer hos de aktuelle pasientene. Feber, blodpropper og betennelse i brusk, lungevev og blodkar er også fellesnevnere, meldte nettstedet Sundhedspolitisk Tidsskrift i sin omtale av saken tidligere i høst.

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  169. Almanya’nın en iyi medyumu haluk hoca sayesinde sizlerde güven içerisinde çalışmalar yaptırabilirsiniz, 40 yıllık uzmanlık ve tecrübesi ile sizlere en iyi medyumluk hizmeti sunuyoruz.

  170. This design is spectacular! You certainly know how to keep a reader amused. Between your wit and your videos, I was almost moved to start my own blog (well, almost…HaHa!) Wonderful job. I really enjoyed what you had to say, and more than that, how you presented it. Too cool!

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  174. Merhaba Ben Haluk Hoca, Aslen Irak Asıllı Arap Hüseyin Efendinin Torunuyum. Yaklaşık İse 40 Yıldır Havas Ve Hüddam İlmi Üzerinde Sizlere 100 Sonuç Veren Garantili Çalışmalar Hazırlamaktayım, 1964 Yılında Irak’ın Basra Şehrinde Doğdum, Dedem Arap Hüseyin Efendiden El Aldım Ve Sizlere 1990 lı Yıllardan Bu Yana Medyum Hocalık Konularında Hizmet Veriyorum, 100 Sonuç Vermiş Olduğum Çalışmalar İse, Giden Eşleri Sevgilileri Geri Getirme, Aşk Bağlama, Aşık Etme, Kısmet Açma, Büyü Bozma Konularında Garantili Sonuçlar Veriyorum, Başta Almanya Fransa Hollanda Olmak Üzere Dünyanın Neresinde Olursanız Olun Hiç Çekinmeden Benimle İletişim Kurabilirsiniz.

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  176. Almanya’nın en iyi güvenilir medyumunun tüm sosyal medya hesaplarını sizlere paylaşıyoruz, güvenin ve kalitelin tek adresi olan medyum haluk hoca 40 yıllık uzmanlığı ile sizlerle.

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  183. Another thing I’ve noticed is the fact for many people, a bad credit score is the results of circumstances past their control. By way of example they may have been saddled having an illness so they have excessive bills going to collections. It could be due to a occupation loss or maybe the inability to go to work. Sometimes divorce or separation can send the funds in the undesired direction. Many thanks for sharing your thinking on this weblog.

  184. Ünlülerin tercih ettiği medyum hocamıza dilediğiniz zaman ulaşabilirsiniz, medyum haluk hocamız sizlerin daimi yanında olacaktır.

  185. Birincisi güvenilir medyum hocaları bulmak olacaktır, ikinci seçenek ise en iyi medyumları bulmak olacaktır, siz hangisini seçerdiniz.

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  209. Thanks for your article. It is rather unfortunate that over the last decade, the travel industry has already been able to to fight terrorism, SARS, tsunamis, bird flu, swine flu, as well as first ever entire global recession. Through it all the industry has proven to be solid, resilient plus dynamic, discovering new strategies to deal with hardship. There are constantly fresh complications and opportunities to which the marketplace must once more adapt and answer.

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