TFAM knockout mice exhibit respiratory chain deficits and reduced mtDNA in midbrain DA neurons, whereas overexpression of TFAM elicits an increase in mtDNA and mitochondrial respiratory chain proteins.
In idiopathic PD, mitochondrial complex I impairment leading to free radical generation which evokes respiratory chain deficits is very well documented [ 6].
To determine whether the respiratory chain has intrinsic deficits, we examined a key mitochondrial enzyme, CcO.
The department store chain had a pensions deficit of £571m after the Green family withdrew hundreds of millions of pounds in dividends.
Lentiviral transduction of cells with wildtype FARS2 restored complex IV protein levels, confirming that the p.Asp325Tyr mutation is pathogenic, causing respiratory chain deficiency and neurological deficits on account of defective aminoacylation of mt-tRNAPhe.
The obvious question for Green and one that is at the heart of the BHS scandal, which has put 11,000 jobs at risk and left the department store chain with a £571m pension deficit.
Sir Philip Green, who used to own BHS, needs to release security he holds over BHS assets in order for the loan to go through, while the Pension Protection Fund is also in talks with the chain over its £571m pension deficit.
Furthermore, there is strong evidence that a deficit in respiratory chain function is involved in the pathogenesis of PD [30], [31].
At the intracellular level, ER stress, mitochondrial respiratory chain deficiency and an autophagy deficit also induce FGF21 expression [ 34- 37].
These results are similar to the phenotype reported for Sprawling (Swl/+) mice, another hypomorphic mutation in dynein heavy chain 1 that exhibits proprioceptive deficits and loss of proprioceptive neurons [13].
