Lipophilic cations like triphenylphosphonium are easily able to penetrate lipid bilayers because they possess a charge that is distributed over a large surface region and the potential gradient allows them to accumulate in the mitochondrial matrix
This allows the uncoupling of ATP synthesis from the proton electrochemical gradient via a proton leak into the mitochondrial matrix
, promoting a decreased [DELTA][PSI] without ATP production (Slocinska et al.
Since initial steps of testosterone, progesterone, and estrogen biosynthesis take place in the mitochondrial matrix
(Felty and Roy 2005; Ramalho-Santos and Amaral 2013), a disruption in mitochondrial integrity or function could possibly lead to endocrine disruption.
In particular, mtDNA single-nucleotide polymorphism A8701G was identified altering mitochondrial matrix
pH and intracellular calcium dynamics and suspected to be involved in pathogenesis of diseases.
29) showed that the Ala-Mn-SOD/mitochondrial targeting sequence allows efficient Mn-SOD import into the mitochondrial matrix
, while the Val-variant causes partial arrest of the precursor within the inner membrane and decreased formation of the active Mn-SOD tetramer in the mitochondrial matrix
Tumor suppressor protein p53 is accumulated in the mitochondrial matrix
and interacts with Cyp-D during ischemia/reperfusion injury.
This is a mitochondrial chaperonin accountable in transportation and protein refolding from the cytoplasm into the mitochondrial matrix
(Deocaris et al.
It is due to deficiency of the pyridoxal phosphatedependent mitochondrial matrix
enzyme ornithine aminotransferase (OAT) which results in hyperornithinaemia to levels 10-15fold above normal.
These 2 structures showed different sizes, and the larger one exhibited a dense crystalloid in the mitochondrial matrix
in the region opposite the axoneme (Fig.
In addition, CoQ10 also allows protons to be extruded from the mitochondrial matrix
to the intermembrane space (3), acts as a pro- or antioxidant (4), and has a role in pyrimidine biosynthesis (5) and apoptosis modulation (6).
The underling mechanism may be that the weak base tacrine is protonated in the acidic intermembrane space of mitochondria and is electrophoretically transported into the mitochondrial matrix
to release a proton in the alkaline matrix and uncouple mitochondrial respiration.
Acetylcarnitine is produced by the mitochondrial matrix
enzyme, carnitine acetyltransferase (CrAT).