PROGETTO AUTOFINANZIATO "BASI MOLECOLARI DELLA ANEMIA SIDEROBLASTICA CONGENITA" (DSB.AD006.322)
Area tematica
Area progettuale
Biologia Molecolare/Cellulare (DSB.AD006)Struttura responsabile del progetto di ricerca
Istituto di Biomembrane, Bioenergetica e Biotecnologie Molecolari (IBIOM)
Responsabile di progetto
SERGIO GIANNATTASIO
Telefono: 080/55929818
E-mail: s.giannattasio@ibiom.cnr.it
Abstract
During the SIR2015 project we realize a model to study the congenital sideroblastic anemia(CSA) caused by mutation in the gene of SLC25A38.
We used P strain to test its phenotype and biochemical parameters. In order to assess a preclinical in vitro characterization for the treatment of CSA, we examined several chemical compounds and their effects on the strain created. With PLP we found the increase in PPIX and Hemin content a significant reduction in ROS content and finally an increase in basal and maximum respiration.
In collaboration with Dott. Iolascon of Ceinge Naples we will we examine several chemical compounds and their effects on the patients cells with CSA caused by mutation in the gene SLC25A38
Data inizio attività
14/04/2021
Parole chiave
slc25a38, sideroblastic anemia, mitochondrial carrier
Ultimo aggiornamento: 15/10/2024