As mice are notorious for their non-humanized lipid profile, especially abundant HDL-C, and that LGMD2B patients have lower HDL-C, the hypothesis of this study was that introducing CETP and its optimal adapter protein human ApoB, a key constituent of nonHDL particles, rather than mouse ApoB, in Dysf mice would reduce HDL-C, increase nonHDL-C and exacerbate MD severity similar to that observed in Dysf/ApoE mice. Here, CETP is linked to Menkes disease.