User:Student100000/Ferritin light chain

Iron is extremely important in the development of neurons, transport through iron-sulfur clusters, the electron transport chain, and synthesis and breakdown of neurotransmitters.The function of the FTL is to act as both an iron reservoir and to remove excess iron from the body. Since iron plays a role in electron transfer, there is potential for the generation of free, highly toxic radicals which makes the role of the FTL as an iron detoxifier very significant.

Oxidative stress caused by iron radicals generated in the ETC and an increase in iron levels has been known to be a cause of the onset of neurodegenerative diseases.

The rates of iron uptake and release may be affected by changes to the components of the ferritin light chains and heavy chains.

An oxygen molecule acts as the terminal electron acceptor during the oxidation of iron in aerobic metabolism. A study conducted with different apoferritins with distinct compositions of heavy and light subunits revealed that both subunits have key roles in the electron transport chain. Neither subunit on its own has the ability to reduce Cytochrome c and thus the first step, the oxidation of Fe2+ to Fe3+, can be carried out by the heavy chain and the light chains are responsible for the transfer of electrons.

FTL is regulated by iron and with an increase in iron, there is both an increase in the FTL expression and PEN-2 levels, which results in increased γ- secretase activity. In relation to this, the downregualtion of FTL expression leads to a decrease in the protein levels on PEN-2.

Mutations in exon four of the FTL gene have been associated with the onset of neuroferritinopathy. There are two distinct toxic mechanisms that lead to neuroferritinopathy and these are abnormalities in iron metabolism and the creation of free iron radicals, resulting in oxidative stress.