Anti-TMLHE Antibody

Primary antibodies raised against trimethyllysine hydroxylase epsilon are central tools for laboratories studying mitochondrial carnitine biosynthesis and X-linked metabolic disorders. This unconjugated rabbit polyclonal reagent from Antibodies.com is supplied as 100 µl of liquid, with reactivity directed at the human TMLHE protein and validation for immunofluorescence (IF) workflows on fixed cells and tissue sections.

TMLHE encodes a mitochondrial enzyme that performs the first committed step of the carnitine biosynthesis pathway by hydroxylating trimethyllysine to hydroxytrimethyllysine, ultimately supporting the production of L-carnitine. L-carnitine shuttles activated long-chain fatty acids into the mitochondrial matrix for beta-oxidation. Because the gene maps to the X chromosome at the Xq28 locus, TMLHE expression patterns and loss-of-function variants have been the subject of substantial research into male-biased phenotypes, including studies examining links between carnitine status and neurodevelopment.

Produced in rabbit hosts and purified as IgG, the reagent is unconjugated, allowing investigators to pair it with secondary antibodies or custom haptens appropriate to their detection platform. The polyclonal format improves the chance of recognizing multiple epitopes on the human target, supporting consistent labeling in indirect fluorescence imaging experiments. Researchers commonly pair such antibodies with mitochondrial markers such as Tom20 or COX IV to confirm subcellular localization, though co-staining should be empirically optimized for the cell type of interest.

Intended for research use only, the product should be handled as a temperature-sensitive protein reagent. Aliquoting upon first thaw helps limit repeated freeze-thaw cycles that can compromise antibody binding, and refrigerated storage preserves activity across the working shelf life of the vial. Each 100 µl pack provides enough material for small-scale IF runs depending on dilution chosen by the user.