- acid
transport across the
mitochondrial membrane by
forming long-chain
acylcarnitine esters which are
shuttled into the mitochondria,
where they undergo...
- Carnitine-
acylcarnitine translocase deficiency is a rare,
autosomal recessive metabolic disorder that
prevents the body from
converting long-chain fatty...
-
palmitoyltransferase II) and
transported back
outside so the
process can
occur again.
Acylcarnitines like
palmitoylcarnitine are
produced as
intermediate products of the...
- The
enzyme acylcarnitine hydrolase (EC 3.1.1.28)
catalyzes the
reaction O-
acylcarnitine + H2O ⇌ {\displaystyle \rightleftharpoons } a
fatty acid + L-carnitine...
- Carnitine-
acylcarnitine translocase (CACT) is
responsible for p****ive
transport of
carnitine and carnitine-fatty acid
complexes and
across the
inner mitochondrial...
- an attack, low
levels of
carnitine in the
blood and high
levels of
acylcarnitine in
blood and
urine may
indicate a
lipid metabolism defect, but these...
-
after birth, by the
analysis of
blood spots collected on
filter paper.
Acylcarnitine profiles with MS/MS will show a very
characteristic pattern of elevated...
-
currently in
clinical trials is danavorexton.
Abnormally low
levels of
acylcarnitine have been
observed in
people with narcolepsy.
These same low levels...
-
Cello C,
Amory H, Marcillaud-Pitel C,
Richard E, et al. (August 2017). "
Acylcarnitines profile best
predicts survival in
horses with
atypical myopathy". PLOS...
- of
orotic acid, as well as rule out an
organic acidemia) and
plasma acylcarnitines (will be
normal in OTC deficiency, but can
identify some
other causes...
