Endothelin-converting enzyme-like 1 (ECEL1) is a putative zinc metalloprotease that was recently identified on the basis of its strong similarity to endothelin-converting enzyme 1. The physiological function of ECEL1 remains unknown so far; the failure to identify a substrate for ECEL1 could be related to the endoplasmic reticulum subcellular localization found by immunofluorescence in recombinant systems. However, clues to the function of ECEL1 were provided by the in-activation of its gene in mice, which resulted in neonatal lethality. The phenotype of homozygous ECEL1 mice, together with the very specific expression profile of its mRNA in the central nervous system, suggests that ECEL1 is crucially involved in the nervous control of respiration.

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