ELOVL1 (ELOVL fatty acid elongase 1) is an endoplasmic reticulum-bound enzyme that catalyzes the first and rate-limiting step in very long-chain fatty acid (VLCFA) biosynthesis 1. The enzyme exhibits highest activity toward C22:0 acyl-CoA substrates and participates in producing both saturated and monounsaturated VLCFAs essential for membrane lipids and sphingolipid synthesis 1. ELOVL1 functions through a four-reaction elongation cycle that adds 2 carbons per cycle to long-chain fatty acids, with particular importance for C24:0 and C24:1 sphingolipid production 1. Disease relevance is significant, as biallelic ELOVL1 variants cause a recognizable syndrome of hypomyelinating leukodystrophy, ichthyosis, progressive spasticity, and complex movement disorders including head tremor and myoclonus 2. Patients show reduced plasma VLCFA concentrations and defective VLCFA synthesis in fibroblasts 2. Clinically, ELOVL1 represents an attractive therapeutic target for X-linked adrenoleukodystrophy, with fibrate compounds like bezafibrate specifically inhibiting the enzyme 1. Additionally, ELOVL1 modulation shows promise in cancer immunotherapy, where its inhibition enhances CD8+ T cell anti-tumor responses by improving metabolic fitness 3, and in treating 1-deoxysphingolipid-associated neuropathies 4.