Biochimica et Biophysica Acta, 1189 (1994) 65-73 65
© 1994 Elsevier Science B.V. All rights reserved 0005-2736/94/$07.00
BBAMEM 76178
The reconstituted carnitine carrier from rat liver mitochondria:
evidence for a transport mechanism different from that
of the other mitochondrial translocators
Cesare Indiveri a,b, Annamaria Tonazzi a and Ferdinando Palmieri a,,
a Department of Pharmaco-Biology, Laboratory of Biochemistry and Molecular Biology, University of Bari, Traversa 200 Re David, 4,
70125 Bari (Italy) and b CNR Unit for the Study of Mitochondria and Bioenergetics, Bari (Italy)
(Received 8 June 1993)
Key words: Mitochondrion; Reconstitution; Carnitine carrier; Transport mechanism;(Rat liver)
The transport mechanism of the reconstituted carnitine carrier purified from rat liver mitochondria was investigated kinetically.
The half-saturation constant (K m) for carnitine on the internal side of the liposomal membrane (8.7 mM) was found to be much
higher than that determined for the external surface (0.45 mM). The exclusive presence of a single transport affinity for carnitine
on each side of the membrane indicated a unidirectional insertion of the carnitine carrier into the proteoliposomes, most
probably right-side-out with respect to mitochondria. Under these defined conditions bisubstrate initial velocity studies of
homologous (carnitine/carnitine) and heterologous (carnitine/acylcarnitine) antiport were performed by varying both the
internal and external substrate concentrations. The kinetic patterns obtained showed that the ratio Km/Vma x is not influenced by
the second (non-varied) substrate, which indicates a ping-pong mechanism. The carnitine carrier thus differs from all other
mitochondrial carriers analyzed so far in the reconstituted state, for which a common sequential type of reaction mechanism has
been found.
Introduction
The inner mitochondrial membrane contains a spe-
cific carrier system for the transport of carnitine and
acylcarnitines, known as the carnitine carrier (for re-
view, see Ref. 1). This system plays a central role in the
translocation of fatty acids via acylcarnitines into the
mitochondrial matrix, where the acyl groups are re-
leased to be used for fatty acid oxidation. The proper-
ties of the carnitine carrier have been extensively inves-
tigated in intact mitochondria [2-10]. This carrier cat-
alyzes a 1 : 1 exchange between acylcarnitines and car-
nitine, which is inhibited by SH reagents like mersalyl
and N-ethylmaleimide and by substrate analogues like
sulphobetaines. Furthermore, besides the exchange re-
action a unidirectional transport of carnitine is cat-
alyzed with lower activity. Recently, we have identified
and purified the carnitine carrier protein from rat liver
mitochondria [11]. When functionally reconstituted, the
purified protein with an apparent molecular mass of
32.5 kDa mediated the exchange reaction between
* Corresponding author. Fax + 39 80 242770.
Abbreviations: DTE, dithioerythritol; Pipes, 1,4-piperazinediethane-
sulphonic acid.
carnitine and acylcarnitines, as well as the uniport
mode of transport [12,13]. The substrate specificity and
inhibitor sensitivity were similar to those found in
intact mitochondria [11-13]. In addition, in the recon-
stituted system we were able to discriminate two classes
of SH-groups that are functionally important for the
transport activity of the carnitine carrier protein [14].
Mainly based on studies in reconstituted systems it
has been proposed that the mitochondrial carriers,
besides forming a structural family [15-17], also consti-
tute a homogenous functional group of proteins, char-
acterized by a common sequential type of reaction
mechanism [1]. For a detailed functional characteriza-
tion of the reconstituted carnitine carrier, and for
comparison with other members of the mitochondrial
carrier family, it is important to establish the orienta-
tion of the inserted protein. Moreover, investigation of
the transport mechanism is of particular interest in the
case of the carnitine carrier since it is the only known
mitochondrial transport system which is able to cat-
alyze both electroneutral exchange and uniport in a
non H+-compensated manner. In this paper, by apply-
ing the two-substrate kinetic analysis, it was possible to
determine whether one (ping-pong mechanism) or two
binding sites (sequential mechanism) have to be occu-
SSDI 0005-2736(93)E0288-L