Tsallake zuwa content

Yin maganin JEB tare da furotin laminin

Mayar da furotin da ya ɓace daga fatar JEB zai iya hana kumburin fata da kuma inganta warkar da rauni.

Wani mutum mai gajeren gashi mai launin ruwan kasa da gemu, sanye da rigar ja a kan farar rigar kwala, ya tsaya a gaban tsaka tsaki.

Dokta Matthew Caley yana aiki a Jami'ar Sarauniya Mary, London, Birtaniya akan wannan aikin don ganin ko za a iya sadar da furotin laminin da ake samu a cikin fata na JEB kuma ya dawo da shi ga lafiya. Ana haifar da alamun JEB lokacin da canje-canje ga girke-girke na kwayoyin halitta na laminin na nufin mutum ba zai iya yin isasshen furotin lamini na kansa ba. Wannan aikin yana da nufin gano ko zai yiwu a maye gurbin furotin ta hanyar allura shi cikin fata mai lafiya ko amfani da shi a cikin maganin gel akan fata mai tabo kuma, idan haka ne, ko wannan maganin zai iya ƙarfafa fatar JEB kuma ya hana kumburi.

 

Game da kudaden mu

 

Jagoran Bincike Dr Matthew Caley
Institution Jami'ar Queen Mary London (QMUL), Birtaniya
Nau'in EB JEB
Hanyar haƙuri Babu
Adadin kuɗi £177,304.39 tare da DEBRA Ireland
Tsawon aikin 2 shekaru
Fara kwanan wata 3 Yuni 2024
DEBRA ID na ciki GR000027

 

Bayanan aikin

Domin 2025.

Jagoran bincike: Dr Matthew Caley babban malami ne a cikin Halittar Halittu (Cibiyar Blizard) tare da gogewa fiye da shekaru goma a cikin binciken fata, ilimin halittar matrix da kuma samar da samfuran fata a cikin vitro. A halin yanzu yana gudanar da gwaje-gwaje na asibiti da aka mayar da hankali kan nau'ikan cututtukan fata daban-daban ciki har da JEB.

Masu bincike: Dokta Emanuel Rognoni babban malami ne a Cibiyar Blizard, QMUL. A lokacin karatun digirin digirgir ya mai da hankali kan nau'in nau'in KEB inda ya bayyana wani sabon aiki na nau'in furotin mai ɗaure Kindlin-1 don homeostasis na epithelial stem cell. Ya ci gaba da ƙware a cikin binciken fata, yana bincikar yadda ƙungiyoyin fibroblast dermal daban-daban ke tsarawa da tasiri juna yayin haɓakawa da warkar da rauni a cikin Lab ɗin Farfesa Fiona Watt (KCL). Yin amfani da fasahohin jeri-fadi na genome, sabbin dandamali na al'adun 2D / 3D da samfuran transgenic / cuta, ƙungiyarsa yanzu tana buɗe hanyoyin ƙwayoyin ƙwayoyin cuta da tasirin fibroblast iri-iri a cikin lafiyar fata da cuta.

Masu haɗin gwiwa: BioLamina.

"Tsarin mu yana da yuwuwar magance blisters cikin sauri da dawo da lafiyar fata, inganta rayuwar rayuwa da rayuwa musamman na marasa lafiya, inda sauran jiyya ba za su yuwu ba."

- Dr Matthew Caley

Sunan bayar: Maganin JEB Tare da Recombinant Laminin 332

Babu magani ga JEB, kula da cutar yana mai da hankali kan sarrafa blisters, sarrafa cututtuka da hana rikitarwa. Tushen cutar shine asarar furotin laminin-332 daga fata. Idan za mu iya maido da laminin-332 za mu iya hanzarta warkar da raunuka da kuma dawo da fata mai koshin lafiya a cikin marasa lafiya na JEB suna inganta rayuwarsu. Bayananmu sun nuna cewa haɓaka laminin-332 na roba yana da yuwuwar inganta alamun cutar JEB.

Wannan aikin yana mayar da hankali kan gano mafi kyawun hanyoyin da za a sadar da laminin-332 a cikin fata na marasa lafiya na JEB don ingantaccen gyara. Yin amfani da samfurin mu na musamman na musamman na JEB za mu gwada hanyoyin bayarwa daban-daban, alluran da za su amfana da fata mara kyau amma cikin sauƙin lalacewa da kuma gel ɗin da za ta amfana da fata mai tabo. Za mu samar da bayanai da ke nuna yadda tartsatsin gyaran lamininmu yake, tsawon lokacin da yake dadewa da kuma nuna tasiri akan tsarin fata da warkar da raunuka.

Don wannan aikin muna haɗin gwiwa tare da kamfanin Sweden BioLamina wanda ya ƙware a cikin samar da laminins don amfani da kimiyya da asibiti. Idan gwaje-gwajenmu na yau da kullun sun yi nasara wannan haɗin gwiwa na musamman zai ba mu damar ciyar da wannan aikin gaba cikin sauri zuwa haƙuri. Ƙaddamar da laminin-332 na roba na iya zama ba magani mai warkarwa ba, duk da haka, tsarinmu yana da damar yin maganin blisters da sauri da kuma mayar da lafiyar fata, inganta yanayin rayuwa da rayuwa musamman ma ƙananan marasa lafiya, inda sauran jiyya ba za su yiwu ba.

Junctional epidermolysis bullosa (JEB) cuta ce ta fata da ba kasafai ba wanda ke haifar da kumburin fata da rauni da rauni na fata, mafi mahimmancin shingen kariya. Wannan yana faruwa ne ta hanyar asarar mahimman sunadaran da ke ɗaure saman saman fata zuwa sauran jikin. Mafi tsanani nau'i, JEB mai tsanani, yana faruwa ta hanyar cikakkiyar asarar ɗaya daga cikin sassan laminin-332 wani mahimmin ɓangaren wannan tsarin. Yaran da aka gano suna da wannan nau'i na JEB kusan duk suna mutuwa kafin cikarsu ta biyu saboda rashin aiki da fatar jikinsu. Marasa lafiya tare da JEB suna da rauni mara kyau kuma suna haɓaka ƙwayar rauni mai kauri a cikin raunukan da ke wanzu waɗanda suka kasa warkewa, suna ƙara haɗarin kamuwa da cutar jini (sepsis). Saboda girman raunukan wannan mummunan nau'in EB yana da zafi sosai.

Ya zuwa yanzu babu wasu ingantattun magunguna da za su iya dakatarwa ko warkar da wannan cuta mai kisa ta fata. Tare da haɗin gwiwar BioLamina muna ba da shawarar gwada idan laminin roba (wanda BioLamina ke samarwa) zai iya inganta warkar da raunuka da kuma hana kumburin fata a cikin tsarin mu na JEB. Za mu gwada hanyoyi daban-daban don sadar da laminin cikin fata (Aim-1), ƙayyade tsawon lokacin da ya rage a cikin fata (Aim-2) kuma idan ya inganta ƙarfin fata (Aim-3) da kuma warkar da rauni (Aim-4). Manufarmu ita ce inganta fatar marasa lafiya na JEB, inganta warkar da raunuka don haka rage haɗarin cututtuka da za su iya haifar da sepsis da mutuwa da wuri.

Domin 2025.