Imikhiqizo

I-BBS4 ingelinye lamaprotheni amaningana ukukhubazeka kwawo kubangela i-Bardet-Biedl syndrome (BBS), ukuphazamiseka kwesistimu ehlukahlukene, ebonakala ngokukhuluphala okuphawulekayo.I-BBS4 polymorphisms ihlotshaniswe nokukhuluphala okuvamile okungeyona i-syndromic morbid.Izindlela zamamolekyuli zokukhuluphala kwe-BBS4, kanye nendima yofuzo lwe-BBS4 ekuhlukaniseni nasekusebenzeni kwe-adipocyte akwaziwa ngokuphelele.Manje sibonisa ukuthi i-Bbs4 idlala indima eqondile nebalulekile ekwandeni kanye ne-adipogenesis: ukuthuliswa kwe-Bbs4 ku-3T3F442A preadipocyte kwenze ukuhlukaniswa kwamaseli okusheshisiwe kanye nokwehlukana okuphambene, okubonakala ngezifundo ze-morphologic (ukukhanya, ukuskena kanye nokudlulisa i-electron microscopy), ukuhlaziywa kwe-fat, iphrofayili ye-fatty acid kanye ne-lipolysis) kanye nemibhalo yezimpawu ze-adipogenic (Cebpα, Pparγ, aP2, ADRP, Perilipin).Kuyo yonke i-adipogenesis nalapho ephonselwa inselelo ngomthwalo wamafutha, amaseli athulisiwe e-Bbs4 aqongelela ama-triglycerides amaningi kakhulu kunokulawula ama-adipocyte, nakuba ngamaconsi amancane (kodwa amakhulu kunani) aqukethe amaphrofayili e-fatty acid eguquliwe.Ngakho-ke, ukunqwabelana kwamafutha amaningi kumaseli athulisiwe kuwumphumela wakho kokubili izinga eliphezulu lokwanda kwe-adipocyte kanye nokuhlukaniswa okuphambene okuholela ekuqongeleleni okungavamile kwamafutha ngeseli ngalinye.Okutholakele kwethu kusikisela ukuthi ukukhuluphala kwe-BBS kungenzeka ngokwengxenye kungenxa yendima eqondile ye-BBS4 ezindleleni ze-physiological and pathophysiological ezingaphansi kokwakheka kwezicubu ze-adipose ezihambisana nokukhuluphala.