MARS1 and Langer mesomelic dysplasia: The functional relevance of CCR4-NOT in LMD was demonstrated by observations that (1) downregulation of LC3B, CNOT1, or CNOT7 increased the relative levels of RLuc-P3′-WT reporter mRNA and endogenous LMD substrates (PRMT1 mRNA and MARS1 mRNA; Fig. 6f), and (2) Rapa treatment shortened the length of poly(A) of endogenous LMD substrates (COTL1 mRNA and CCT7 mRNA) in a way that was reversed by LC3B downregulation.