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All indirect calorimetric measurements at 36°C and below were performed with concomitant sedation, analgesia and NMB.

Unless otherwise indicated, all experiments reported below were performed with RBCs from the index patient.

Transient transfections of wt and mutant hRFC constructs (see below) were performed with Lipofectamine™ Plus reagent (Invitrogen), as described previously [ 15, 16].

On the basis of cellular toxicity results, all the experiments described below were performed with HEMA (4.0 mmol/L), or TEGDMA (0.7 mmol/L), or UDMA (0.2 mmol/L), or BDDMA (0.4 mmol/L), or DMSO 0.1%, or culture medium (control) and the incubations were performed in a humidified atmosphere of 5% CO2.

As our protocol consistently yielded stronger and more distinct staining for all the antibodies, the final staining for all the 175 cases with SMA and a double immunostaining with additional markers in selected cases (see below) were performed with our published protocol [ 26].

Although there was a difference in activity between the two epimers, the biological results using a 1 1 epimeric mixture (in which the concentration of the more active epimer is halved) proved to be similar to those using the single epimer (S)- 10, and for this reason, all subsequent experiments described below were performed with the more readily accessed epimeric mixture.

Quantification of bands on western blots (using the antibodies below) was performed with Image J software (version 1.39 u).

All of the statistical tests described below, except one, were performed with algorithms in Mathematica available through reference [31].

However, due to practical limitations mentioned in our discussion of additional tests below, most tests were performed with the model that uses a constant K.

Two experimental series, below called enhancing and suppressing, were performed with the test MRP concentration of 0.0025%, 0.005%, 0.01%, 0.025%, and 0.1% in 0.08 M NaCl.

The analysis described below is performed on the isochore sequences with masked repeats.