Kwayoyin tsarin rigakafi da RDEB raunuka (2022)

Covid ya katse wannan aikin sosai, don haka masu binciken suka haɓaka wata hanya ta gwaji don magance ainihin tambayoyin bincike. Suna bayar da rahoton ci gaba mai mahimmanci wajen fahimtar yadda tsarin rigakafi ke shiga cikin aikin fata kuma sunyi imani da binciken da suka buga a cikin labaran bincike masu zuwa zasu kasance da amfani ga marasa lafiya tare da RDEB:

• Epidermal mammalian manufa na hadaddun rapamycin 2 yana sarrafa haɓakar lipid da sarrafa filaggrin a cikin samuwar shinge na epidermal - Jaridar Allergy da Clinical Immunology 
• Metabolism na Glutamine yana nuna kararraki Stace Celet Fasali mai juyawa da dogon lokaci a cikin gashi follicle - cel metabolic
• Ƙa'idar Amsa Warkar da Rauni a lokacin tsufa - Jaridar Bincike na Dermatology
• Mitochondrial metabolism yana daidaita matakan ƙayyadaddun matakan gyaran matakai a cikin macrophages yayin warkar da rauni - Metabolism Cell
• Matsayin mTOR Signaling Cascade a cikin Epidermal Morphogenesis da Samuwar Barrier Skin 

Jagoran bincike:

Sabine Eming: Farfesa da Jagoran Likita a Dept. na Dermatology, Jami'ar Cologne, yana jagorantar shirin aiki a cikin lalacewar nama da gyare-gyaren da ke tattare da kewayon daga tsarin bincike na asali-aiki, ta hanyar a cikin vivo model, zuwa cutar mutum. Ƙungiyarta tana da sha'awar fahimtar yadda fata ke jin lalacewar nama da kuma yadda waɗannan abubuwan da suka faru suka juya zuwa amsawar farfadowa, samuwar tabo ko cuta. Ɗaya daga cikin mayar da hankali na ƙungiyar shine rarraba tsaka-tsakin tsaka-tsakin nama na musamman na iya sake farfadowa da kuma amsawar rigakafi. Ita ce Babban Mai binciken ayyukan bincike na ɓangare na uku da yawa da gwaje-gwaje na asibiti, ta bayyana cututtukan cututtuka a cikin cututtuka daban-daban da kuma fassara wannan ilimin zuwa ingantaccen kulawar rauni a cikin marasa lafiya. Za ta daidaita aikin da hulɗa tare da abokan haɗin gwiwa a Freiburg. Za ta kasance da alhakin nasarar nasarar aikin, jagorar ɗalibin PhD a cikin ƙirar gwaji, ƙididdigar kimiyya na sakamakon, rubutun rubutun, da fassarar.

Masu bincike:

Dimitra Kiritsi: Mashawarci likitan fata da Junior Group Leader, ya yi aiki na 9 shekaru a matsayin likita-masanin kimiyya a Sashen Dermatology da EB-Center Freiburg. An haɗa ta a cikin bincike, gudanarwa da kulawa da yawa na marasa lafiya tare da EB. Binciken nata yana mai da hankali kan cututtukan cututtuka da haɓaka sabbin hanyoyin warkewa don cututtukan fata da aka gada, musamman EB da mosaicism na fata. Tana da ƙwarewa sosai a matsayin babban jami'a kuma mai bincike na gwaji a cikin gwaje-gwajen asibiti guda 11 tare da cututtukan blistering (autoimmune da EB). A halin yanzu ita ce Shugabar dakin gwaje-gwaje na Immunofluorescence, "Sashin gwaji na Clinical Skin Skin" da Sashin Kula da Rauni na Dept. Dermatology a Freiburg. Za ta ba da kayan haƙuri da bayanan da ake buƙata don nazarin da aka ba da shawara, kuma za ta ba da gudummawa ga nazarin sakamakon bayan kammala binciken da rubuta labaran da suka dace.

Alexander Nyström: shi ne jagoran rukuni a Sashen Nazarin fata, Cibiyar Kiwon Lafiya - Jami'ar Freiburg. Yana da gogewa mai yawa tare da samfuran RDEB na yau da kullun, kuma zai raba ƙwarewar aikin sa tare da nazarin warkar da rauni.

"A cikin wannan binciken za mu sami kyakkyawar fahimta game da raunin raunin rauni a cikin marasa lafiya na RDEB da kuma yadda za a iya gano matsalolin da suka biyo baya da wuri kuma za a iya hana su."

Farfesa Dr Sabine Ememing

Taken Bayar: Bincika rigakafi na asali a cikin rikice-rikicen warkar da rauni a cikin marasa lafiya RDEB

A cikin wannan tsari shine babban sha'awarmu don gano maƙasudin kwayoyin da ke taka muhimmiyar rawa a cikin rikice-rikice na warkar da raunuka (mafi yawan tabo da carcinogenesis) a cikin marasa lafiya na RDEB da kuma dawo da ilimin da aka samu zuwa ga mai haƙuri don inganta maganin raunin gida da ci gaban cututtuka.

Kwayoyin tsarin rigakafi wani abu ne mai mahimmanci na iyawar jiki don dawo da aikin nama bayan rauni. Bayan yawancin nau'ikan lalacewar nama ciki har da blistering a cikin marasa lafiya na EB, takamaiman ƙwayoyin rigakafi musamman monocytes/macrophages suna yin ayyuka masu mahimmanci guda biyu: don ba da amsa da sauri ga tsarin ƙwayoyin cuta-da lalata da ke da alaƙa kuma daga baya don taimakawa gyara lalacewar nama.

Wannan tsari yana buƙatar macrophages don fara ɗaukar phenotype pro-inflammatory sannan daga baya lokacin da haɗarin nan da nan ya wuce don samun nau'in phenotype na anti-mai kumburi don haɓaka ƙuduri da gyarawa.

A yanzu ya bayyana a sarari cewa ƙwaƙƙwaran sarrafa ƙarfi tsakanin wannan haɓakar kumburi da ƙudurin kunnawa phenotype a cikin macrophages yana da mahimmanci don ingantaccen amsawar warkarwa. Muna ba da shawara don fallasa rawar macrophages a cikin warkar da rauni a cikin marasa lafiya na RDEB da kuma gano dabarun yadda za a daidaita ayyukan macrophage a cikin raunukan marasa lafiya na RDEB.

Macrophages kuma suna da alaƙa da haɗin gwiwa a cikin samuwar cutar kansa kuma ta hanyar injiniyoyi ana iya sanya su a tsaka-tsaki tsakanin raunin warkarwa mara kyau da ke da alaƙa da RDEB da kuma canza raunin a hankali zuwa carcinoma. Mun yi imani da ƙarfi cewa ta hanyar ƙaddamar da mahimman hanyoyin da ke ba da gudummawar yadda ƙwayoyin rigakafi ke lalata raunin rauni a cikin marasa lafiya na RDEB ba kawai za su ci gaba da haɓaka sabbin mahaɗan warkewa na gida waɗanda ke hanzarta rufe rauni ba (misali suturar rauni wanda ke lalata kumburi na yau da kullun) amma kuma yana iya taimakawa wajen haɓaka kayan aikin bincike don saka idanu lokacin da rauni mara kyau ya zama m.

An fara gwaje-gwaje kamar yadda aka tsara a cikin ainihin shirin aikin. Bugu da ƙari, a cikin layi daya da gwaje-gwajen da aka tsara a cikin shirin farko na aikin aikin, dakin gwaje-gwaje na mai nema ya sami ci gaba mai mahimmanci a fahimtar yadda tsarin rigakafi ke tasiri wajen kiyaye aikin shinge na fata na epidermal, a cikin ayyukan da ke da alaƙa da ci gaba a cikin ƙungiyar mai nema. Mun yi imanin cewa waɗannan binciken za su ba da gudummawa ga ƙa'idar fahimtar yadda rigakafi na asali ke haifar da rikice-rikicen warkar da raunuka a cikin marasa lafiya na RDEB, kuma marasa lafiyar RDEB za su amfana daga waɗannan binciken. Anan muna ganin dama don ingantaccen bincike don amfanin marasa lafiya na RDEB. (Daga rahoton ci gaba na 2019.)

An fara gwaje-gwaje kamar yadda aka tsara a cikin ainihin shirin aikin. Da farko an sami wasu jinkiri wajen samar da takamaiman samfurin kwayoyin halitta, wanda muka yi la'akari da cewa an warware shi a farkon 2020. Duk da haka, ba zato ba tsammani a farkon 2020 an rufe rayuwar ilimi da dakunan gwaje-gwaje na gwaji saboda hana cutar COVID-19 na Jami'ar. A cikin 2020 da 2021 rayuwar jami'a ta tsaya cik. Ba za a iya amfani da kayan aiki da dakunan gwaje-gwaje azaman shiri ba kuma an taƙaita musanya ta kimiyya da magana sosai. Sakamakon waɗannan iyakoki, a harabar nasu amma har ma a duniya (jinkirin yin oda da isarwa daga albarkatun lab, ƙarancin ma'aikata saboda rashin lafiya), har yanzu ba a shawo kan su gaba ɗaya ba. Gabaɗaya, ƙayyadaddun cutar ta yi tasiri sosai ga aikinmu da ainihin shirin aikin. Saboda haka, kamar yadda aka bayyana a cikin rahoton wucin gadi daga 25 Disamba 2019, mai nema ya ɓullo da wata hanya ta gwaji wadda ake sa ran za ta dace da shirin farko na aikin da kuma magance ainihin tambayoyin bincike na aikin. dakin gwaje-gwajen mai nema ya sami babban ci gaba a fahimtar yadda tsarin rigakafi ke tasiri ga kiyaye aikin shingen fata na epidermal, a cikin ayyukan da ke da alaƙa da gudana a cikin ƙungiyar mai nema. Mun yi imanin cewa waɗannan binciken za su ba da gudummawa ga ƙa'idar fahimtar yadda nau'in rigakafi na 2 ke tasiri matsalolin warkar da raunuka a cikin marasa lafiya na RDEB, kuma marasa lafiya na RDEB za su amfana daga waɗannan binciken. Anan muna ganin dama don ingantaccen bincike don amfanin marasa lafiya na RDEB. (Daga rahoton ci gaba na ƙarshe na 2022.)