LZTR1, a member of the BTB-kelch superfamily, was initially described as a putative transcriptional regulator based on weak homology to members of the basic leucine zipper-like family, the encoded protein subsequently has been shown to localize exclusively to the Golgi network where it may help stabilize the Golgi complex. Deletion of this gene may be associated with DiGeorge syndrome, a developmental field defect involving the third and fourth pharyngeal pouches, causing the absence of thymus and parathyroid glands, congenital cardiac abnormalities and facial dysmorphism. LZTR1 is tyrosine phosphorylated and subsequently degraded upon induction of apoptosis.

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