how does giving l carnitine work in primary carnitine deficiency

how does giving l carnitine work in primary carnitine deficiency Suggested Action of L-Carnitine 23

Name: how does giving l carnitine work in primary carnitine deficiency Suggested Action of L-Carnitine 23
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122,123,124 Gln enters the cell via the amino acid transporter ASCT2/SLC1A5 and is converted to glutamate in the mitochondria by a deamination reaction involving glutaminase (GLS)