Question

how can electrophoretic techniques be used to analyse defects in mitochondrial respiratory chain complexes?

Answer 1

Mitochondrial respiratory chain evaluation and oxidative phosphorylation system with the help of blue native gel electrophoresis.

OXPHOS oxidative phosphorylation system are mainly of five types of multimeric complex present in the inner membrane of the mitochondria.

It works for drive the aerobic synthesis of the ATP. mitochondrial diseases occur due to nuclear DNA mutation and mitochondria affects the accumulation and the activities of enzymes and it got affected by aging and neurodegeneration as well.

The above reasons are responsible to develop the oxidative phosphorylation system or OXPHOS.

Blue native gel that is BNGE regularly added the above technique of OXPHOS system and help in screening of enzymatic defects in mitochondrial preparation derived from cultured cells.

BNGE are used oftenly as complement to respiratory chain in studies of Oxygen consumption.

It is actually the separation of protein complexes by the electrophoresis which is based on the weight of native molecular when coomassive blue is present.

Protein complex are derived or extracted through membrane in the presence of detergent used in mild condition and then it is exposed to dye which have high charge used for electrophoric seperation without disturbing the native conformation.

BNGE are used to analyse the mitochondrial extract from yeast or cellular extract and human cell lines or tissue of mouse also diagnostic tool to characterize the electron transport chain derived sample from pateint those are having mitochondrial diseases.