KEB ndi khansa yapakhungu (2024)

Chotsatira 1: Maselo a khansa omwe ali ndi kusintha kwa chibadwa cha KEB (kusowa kwa kindlin-1) awonetsedwa kuti amatha kupanga khansa yachiwiri m'madera ena a thupi.

Chotsatira 2: Maselowa ali ndi mapuloteni apamwamba omwe amakhudzana ndi kufalikira kwa khansa. Kuchepetsa puloteni iyi m'maselo kunachepetsa mphamvu zawo zopanga khansa ndipo izi zikhoza kukhala maziko a chithandizo chamtsogolo cha squamous cell carcinoma mu EB.

Chotsatira 3: Zotsatira zoyamba zimasonyeza kuti kuwala kwa UV kumawonjezera mapuloteni omwe amakhudzidwa ndi kutupa m'maselo a khansa. Imawonjezera makulidwe a khungu (epidermis) ndi kuchuluka kwa ma cell akhungu (keratinocytes) ofanana ndi chiyambi cha kukula kwa khansa.

Ntchito imeneyi inali lofalitsidwa m’magazini yotchedwa Oncogenesis mu 2024. Zinaperekedwa muzokambirana ku Msonkhano Wapachaka wa British Society for Investigative Dermatology mu 2022 ndi Msonkhano wa 99th Scottish Skin Biology Club mu 2023. Komanso, mu chiwonetsero chazithunzi pa 1st Edinburgh Skin Network Symposium mu 2023.

Kusintha kwamavidiyo a 2024:

Mtsogoleri wa kafukufuku:

Pulofesa Valerie Brunton ndi Wapampando wa Cancer Therapeutics ku yunivesite ya Edinburgh. Zokonda zake ndikumvetsetsa biology ya Kindler EB yokhudzana ndi atrophy yapakhungu (kufooka), photosensitivity, komanso chiwopsezo chowonjezereka chokhala ndi khansa yamtundu wa squamous cell. Kafukufuku wake akufuna kudziwa njira zazikulu zama cell zomwe zimayambitsa matenda a Kindler EB kuti athandizire kuzindikira njira zomwe angachitire izi.

Ofufuza nawo:

Dr Adam Byron (biochemistry) University of Edinburgh and Prof Albena Dinkova-Kostova (cellular medicine) University of Dundee.

Wothandizira:

Dr Alan Serrels (khansa / chotupa microenvironment) University of Edinburgh.

"Kafukufuku wathu wapeza kusintha kwakukulu kwa chotupa microenvironment (maselo abwinobwino ndi mamolekyu omwe amazungulira ndikuthandizira kukula kwa maselo otupa), omwe amayendetsedwa ndi Kindlin-1. Pophunzira kusintha kumeneku, tikuyembekeza kumvetsetsa bwino momwe tingapewere kukula ndi kukula kwa khansa yapakhungu mwa odwala omwe ali ndi matenda a Kindler. "

- Prof Valerie Brunton

Grant Title: Kumvetsetsa udindo wa Kindlin-1 kutayika pakukula kwa squamous cell carcinoma

Kindler EB (KEB) ndi matenda osowa khungu omwe amayamba adakali aang'ono. Anthu omwe ali ndi KEB amakhala ndi matuza pakhungu ndipo amakhala ndi khungu lopyapyala kapena lamapepala. Amakhudzidwanso kwambiri ndi cheza cha ultraviolet (UV) kuchokera kudzuwa komanso kupsa ndi dzuwa mosavuta. KEB imawonjezeranso chiopsezo chokhala ndi mtundu wa khansa yapakhungu yotchedwa squamous cell carcinoma. KEB imachitika chifukwa cha kusintha kwa majini mu jini ya FERMT1. Kusintha kwa masinthidwe mu FERMT1 kumapangitsa kupanga puloteni yolakwika yotchedwa Kindlin-1.

Pakadali pano sizidziwika bwino chifukwa chake kutayika kwa puloteni yogwira ntchito ya Kindlin-1 pakhungu la anthu omwe ali ndi KEB kumawonjezera chiopsezo chokhala ndi squamous cell carcinoma (SCC). Gululo lidachitapo zoyeserera pama cell akhungu omwe adakula mu labotale zomwe zawonetsa kuti kutayika kwa Kindlin-1 kumapangitsa kuti maselo azitha kumva kuwala kwa UV. Kuti amvetse chifukwa chake izi zimachitika adzagwiritsa ntchito chitsanzo chomwe chimatsanzira kukhudzana ndi dzuwa ndi cheza cha UV. Gawo lotsatira liwona ngati kutayika kwa Kindlin-1 pakhungu kumabweretsa kupangika kwa khansa yapakhungu pambuyo pakuwonekera kwa UV.

Zolinga zitatu za polojekitiyi ndi:

1. Kodi kusintha kwamtundu wa zotupa zomwe zilibe Kindlin-1 kumathandizira kukula kwawo komanso kufalikira kwa metastatic?
2. Kodi abwenzi a Kindlin-1 (mamolekyu omwe amalumikizana), amawongolera kukula kwa khansa (SCC)?
3. Kodi kutayika kwa Kindlin-1 kumalimbikitsa mapangidwe a khansa yapakhungu ya UV (SCC)?

DEBRA UK adathandizidwa kale Pulofesa Valerie Brunton ku yunivesite ya Edinburgh ku kafukufuku wa Kindler Syndrome.

Kindler EB (KEB) ndi matenda osowa khungu omwe amayamba adakali aang'ono. Anthu omwe ali ndi KEB amakhala ndi matuza pakhungu, ndipo amakhala ndi khungu lopyapyala kapena lamapepala. Amakhudzidwanso kwambiri ndi cheza cha ultraviolet (UV) kuchokera kudzuwa komanso kupsa ndi dzuwa mosavuta. KEB imawonjezeranso chiopsezo chokhala ndi mtundu wa khansa yapakhungu yotchedwa squamous cell carcinoma. KEB imachitika chifukwa cha kusintha kwa majini mu jini ya FERMT1. Kusintha kwa FERMT1 kumapangitsa kupanga puloteni yolakwika yotchedwa Kindlin-1. Pakadali pano sizidziwika bwino chifukwa chake kutayika kwa puloteni yogwira ntchito ya Kindlin-1 pakhungu la anthu omwe ali ndi KEB kumawonjezera chiopsezo chokhala ndi squamous cell carcinoma. Tidachitapo kale zoyeserera zama cell akhungu omwe amakula mu labotale zomwe zawonetsa kuti kutayika kwa Kindlin-1 kumapangitsa kuti maselo azitha kumva kuwala kwa UV. Kuti timvetse chifukwa chake izi zimachitika tigwiritsa ntchito chitsanzo chomwe chimatsanzira kutenthedwa ndi dzuwa ku kuwala kwa UV. Izi zizindikiritsa zosintha zoyambitsidwa ndi UV zomwe zimayendetsedwa makamaka ndi kutayika kwa Kindlin. Gawo lotsatira liwona ngati kutayika kwa Kindlin-1 pakhungu kumabweretsa kupangika kwa khansa yapakhungu kutsatira kuwonekera kwa UV (Kuyambira 2022 & 2023 malipoti opita patsogolo ndi lipoti lomaliza la 2024).