The mitochondrial oxidation of long-chain fatty acids is initiated by the sequential action of carnitine palmitoyltransferase I (which is located in the outer membrane and is detergent-labile) and carnitine palmitoyltransferase II (which is located in the inner membrane and is detergent-stable), together with a carnitine-acylcarnitine translocase. CPT I is the key enzyme in the carnitine-dependent transport across the mitochondrial inner membrane and its deficiency results in a decreased rate of fatty acid beta-oxidation. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
Inmunógeno
PBS with 0.05% proclin300,0.05% BSA,50% glycerol,pH7.3.
Recombinant fusion protein containing a sequence corresponding to amino acids 520-720 of human CPT1A (NP_001867.2).
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