Gluten Intolerance: Ib Txoj Kev Cia Siab Rau Kev Tsim Kho Kev Kho Mob Cystic Fibrosis thiab Celiac Disease

Txoj kev tshawb no qhia cov protein tshiab koom nrog hauv kev txhim kho gluten intolerance uas tuaj yeem yog lub hom phiaj kho mob.

Yuav luag 1 ntawm 100 tus neeg raug kev txom nyem los ntawm celiac kab mob, ib hom kab mob caj ces uas qee zaum kuj tuaj yeem tshwm sim los ntawm ib puag ncig thiab kev noj haus. Cov neeg mob los ntawm celiac kab mob txhim kho qhov rhiab heev rau gluten - pom nyob rau hauv hom qoob mog, rye thiab barley. Tus kab mob no yog ib qho mob autoimmune loj heev ntawm peb cov hnyuv uas peb lub cev tiv thaiv kab mob ua rau muaj kev teb rau peb lub cev cov hlwb - yog li 'autoimmunity' - thaum twg khoom noj khoom haus muaj gluten noj. Cov lus teb tsis zoo no los ntawm peb lub cev tiv thaiv kab mob ua rau lub ntsej muag ntawm cov hnyuv me. Thaum xub thawj tus kab mob celiac tau pom nyob rau hauv cov teb chaws uas muaj cov neeg Caucasian ntau dua, tam sim no nws kuj tau tshaj tawm hauv cov pej xeem. Hmoov tsis zoo tsis muaj kev kho mob rau cov kab mob celiac thiab cov neeg mob yuav tsum tau ceev faj saib xyuas lawv cov zaub mov uas yog tib txoj kev kho.

Kev sib koom ua ke ntawm kab mob celiac thiab cystic fibrosis

Kab mob celiac kuj tshwm sim ntau dua (los ntawm yuav luag peb zaug) hauv cov neeg uas raug kev txom nyem los ntawm cov leeg nqaij hlav vim tias muaj kev sib koom ua ke ntawm ob kab mob no. Nyob rau hauv cystic fibrosis, tuab thiab nplaum mucus tsim nyob rau hauv lub ntsws thiab txoj hnyuv feem ntau tshwm sim los ntawm kev hloov nyob rau hauv cov noob rau protein CFTR (cystic fibrosis transmembrane conductance regulator). CFTR protein plays lub luag haujlwm tseem ceeb hauv kev ua kom cov kua qaub. Yog li, thaum cov ion thauj cov protein no tsis tsim, cov hnoos qeev pib txhaws thiab qhov ua haujlwm tsis zoo no tseem ua rau lwm yam teeb meem tshwm sim hauv lub ntsws, hnyuv thiab lwm yam kabmob feem ntau yog vim lub cev tsis muaj zog tau qhib. Cov tshuaj tiv thaiv lossis cov teebmeem no zoo ib yam li qhov tshwm sim los ntawm gluten hauv cov neeg mob celiac. Tias yog vim li cas nws thiaj nkag siab tias ob qho kev tsis sib haum xeeb no txuas nrog.

Cov kws tshawb fawb los ntawm Ltalis thiab Fabkis tau teeb tsa kom nkag siab qhov xwm txheej ntawm kev sib txuas ntawm kab mob celiac thiab cystic fibrosis ntawm qib molecular hauv lawv txoj kev tshawb fawb luam tawm hauv EMBO Journal. Txij li thaum gluten yog qhov nyuaj rau zom, nws cov protein ntev dua nkag mus rau hauv cov hnyuv. Cov kws tshawb fawb tau siv tib neeg txoj hnyuv kab mob hauv cov chaw kuaj mob uas nkag siab rau gluten. Nws tau pom tias ib feem ntawm cov protein (lossis peptide) hu ua P31-43 tuaj yeem khi ncaj qha rau CFTR thiab ua rau nws tsis zoo. Thiab ib zaug kev ua haujlwm ntawm CFTR raug cuam tshuam, cellular stress thiab o tau tshwm sim. Cov kws tshawb fawb tau xaus lus tias CFTR yog qhov tseem ceeb hauv kev kho gluten rhiab heev rau cov neeg mob celiac.

Ib qho kev sib xyaw tshwj xeeb hu ua VX-770 tuaj yeem cuam tshuam kev sib cuam tshuam ntawm peptide P31-43 thiab CFTR protein los ntawm kev thaiv qhov chaw nquag ntawm lub hom phiaj protein. Yog li, thaum tib neeg lub plab zom mov lossis cov ntaub so ntswg uas tau sau los ntawm cov neeg mob celiac tau ua ntej incubated nrog VX-770, kev sib cuam tshuam ntawm cov peptide ntxiv thiab cov protein tsis tshwm sim thiab yog li kev tiv thaiv kab mob tsis raug tshem tawm txhua. Qhov no cim VX-770 yog qhov tseem ceeb rau kev tiv thaiv gluten-sensitive epithelial hlwb los ntawm kev tsis zoo ntawm gluten noj. Hauv cov nas gluten rhiab heev, VX-771 muab kev tiv thaiv los ntawm gluten-induced plab hnyuv cov tsos mob.

Txoj kev tshawb no yog ib qho kev cog lus thawj kauj ruam ntawm kev txhim kho kev kho mob los ntawm inhibitors ntawm protein CFTR uas tuaj yeem kho cystic fibrosis thiab kuj tseem tuaj yeem yog qhov pib rau kev tsim kho cov kab mob celiac. Kev sim tshuaj ntau ntxiv yog xav tau los txheeb xyuas cov koob tshuaj thiab kev tswj hwm ntawm cov muaj peev xwm CFTR inhibitors. Cov txiaj ntsig tuaj yeem pab cov neeg mob uas muaj gluten intolerance kom muaj peev xwm siv tshuaj tsis hloov lossis txwv lawv cov zaub mov.

***

{Koj tuaj yeem nyeem cov ntawv tshawb fawb thawj zaug los ntawm nyem rau ntawm DOI qhov txuas tau muab hauv qab no hauv cov npe ntawm cov khoom raug suav nrog}

Qhov (s)

Villella VR et al. 2018. Lub luag haujlwm pathogenic rau cystic fibrosis transmembrane conductance regulator hauv kab mob celiac. EMBO Journalhttps://doi.org/10.15252/embj.2018100101

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