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Pregled bibliografske jedinice broj: 463127

Immune dysregulation in mitochondrial disease


Gagro, Alenka; Karačić, Iva; Horvath, Rita; Ćuk, Mario; Sarnavka, Vladimir; Tešović, Goran; Lochmuller, Hans; Barišić, Nina; Novak, Milivoj; Galić, Slobodan et al.
Immune dysregulation in mitochondrial disease // 6. HRVATSKI KONGRES O INFEKTIVNIM BOLESTIMA s medunarodnim sudjelovanjem, Šibenik
Šibenik, Hrvatska, 2009. (pozvano predavanje, domaća recenzija, sažetak, znanstveni)


CROSBI ID: 463127 Za ispravke kontaktirajte CROSBI podršku putem web obrasca

Naslov
Immune dysregulation in mitochondrial disease

Autori
Gagro, Alenka ; Karačić, Iva ; Horvath, Rita ; Ćuk, Mario ; Sarnavka, Vladimir ; Tešović, Goran ; Lochmuller, Hans ; Barišić, Nina ; Novak, Milivoj ; Galić, Slobodan ; Jelušić, Marija ; Vidović, Mandica ; Begović, Davor ; Tambić-Bukovac, Lana ; Fumić, Ksenija ; Barić, Ivo

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
6. HRVATSKI KONGRES O INFEKTIVNIM BOLESTIMA s medunarodnim sudjelovanjem, Šibenik / - , 2009

Skup
6. HRVATSKI KONGRES O INFEKTIVNIM BOLESTIMA s medunarodnim sudjelovanjem

Mjesto i datum
Šibenik, Hrvatska, 24-27.10.2009

Vrsta sudjelovanja
Pozvano predavanje

Vrsta recenzije
Domaća recenzija

Ključne riječi
OXPHOS; T-cell immunodeficiency; autoimmune reactions

Sažetak
Mitochondria are cytoplasmic organelles in eukaryotic cells that accomplish several distinct vital functions, including oxidative phosphorylation (OXPHOS), metabolic pathways, and integration of signaling for apoptosis. Impaired OXPHOS, the common final pathway of mitochondrial metabolism, results in a variety of clinical manifestations, and the term mitochondrial disorders is currently ascribed to (mostly) genetic diseases of the respiratory chain associated with mitochondrial DNA mutation or nuclear DNA mutations. In addition, dysfunction of mitochondria could also result from three large groups of diseases with mitochondrial impairments: 1) secondary mitochondrial diseases which are provoked by mutations in the non-mitochondrial genome leading to mitochondriotoxic effects by mutated proteins or crucially altered regulatory processes, 2) acute mitochondrial insults due to ischemia, inflammation, and intoxications, and 3) changes of mitochondrial regulation and function in cancer cells. In any case, impaired mitochondrial function leads to cellular energetic depression which is characterized by diminished phosphorylation potentials, cytoplasmic and mitochondrial Ca2+ overload, and accumulation of ROS and toxic proteins. We present the clinical and laboratory features of a girl with combined respiratory chain defect and immunologic impairment that includes T-cell immunodeficiency and autoimmune reactions. In addition, a short overview on the current knowledge of immune system involvement in primary and secondary defects of the mitochondrial respiratory chain will be presented.

Izvorni jezik
Engleski

Znanstvena područja
Temeljne medicinske znanosti, Kliničke medicinske znanosti



POVEZANOST RADA


Projekti:
072-1080229-0337 - Modulacija funkcije ljudskih regulacijskih T-limfocita (Gagro, Alenka, MZOS ) ( POIROT)
108-1081870-1885 - Nasljedne metaboličke i ostale monogenske bolesti djece (Barić, Ivo, MZOS ) ( POIROT)

Ustanove:
Klinika za dječje bolesti Medicinskog fakulteta,
Medicinski fakultet, Zagreb,
Klinika za infektivne bolesti "Dr Fran Mihaljević",
Klinički bolnički centar Zagreb


Citiraj ovu publikaciju:

Gagro, Alenka; Karačić, Iva; Horvath, Rita; Ćuk, Mario; Sarnavka, Vladimir; Tešović, Goran; Lochmuller, Hans; Barišić, Nina; Novak, Milivoj; Galić, Slobodan et al.
Immune dysregulation in mitochondrial disease // 6. HRVATSKI KONGRES O INFEKTIVNIM BOLESTIMA s medunarodnim sudjelovanjem, Šibenik
Šibenik, Hrvatska, 2009. (pozvano predavanje, domaća recenzija, sažetak, znanstveni)
Gagro, A., Karačić, I., Horvath, R., Ćuk, M., Sarnavka, V., Tešović, G., Lochmuller, H., Barišić, N., Novak, M. & Galić, S. (2009) Immune dysregulation in mitochondrial disease. U: 6. HRVATSKI KONGRES O INFEKTIVNIM BOLESTIMA s medunarodnim sudjelovanjem, Šibenik.
@article{article, author = {Gagro, Alenka and Kara\v{c}i\'{c}, Iva and Horvath, Rita and \'{C}uk, Mario and Sarnavka, Vladimir and Te\v{s}ovi\'{c}, Goran and Lochmuller, Hans and Bari\v{s}i\'{c}, Nina and Novak, Milivoj and Gali\'{c}, Slobodan and Jelu\v{s}i\'{c}, Marija and Vidovi\'{c}, Mandica and Begovi\'{c}, Davor and Tambi\'{c}-Bukovac, Lana and Fumi\'{c}, Ksenija and Bari\'{c}, Ivo}, year = {2009}, keywords = {OXPHOS, T-cell immunodeficiency, autoimmune reactions}, title = {Immune dysregulation in mitochondrial disease}, keyword = {OXPHOS, T-cell immunodeficiency, autoimmune reactions}, publisherplace = {\v{S}ibenik, Hrvatska} }
@article{article, author = {Gagro, Alenka and Kara\v{c}i\'{c}, Iva and Horvath, Rita and \'{C}uk, Mario and Sarnavka, Vladimir and Te\v{s}ovi\'{c}, Goran and Lochmuller, Hans and Bari\v{s}i\'{c}, Nina and Novak, Milivoj and Gali\'{c}, Slobodan and Jelu\v{s}i\'{c}, Marija and Vidovi\'{c}, Mandica and Begovi\'{c}, Davor and Tambi\'{c}-Bukovac, Lana and Fumi\'{c}, Ksenija and Bari\'{c}, Ivo}, year = {2009}, keywords = {OXPHOS, T-cell immunodeficiency, autoimmune reactions}, title = {Immune dysregulation in mitochondrial disease}, keyword = {OXPHOS, T-cell immunodeficiency, autoimmune reactions}, publisherplace = {\v{S}ibenik, Hrvatska} }




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