Protein-cysteine N-palmitoyltransferase HHAT

Definition:

Palmitoyl acyltransferase that catalyzes N-terminal palmitoylation of SHH; which is required for SHH signaling. It also catalyzes N-terminal palmitoylation of DHH. Promotes the transfer of palmitoyl-CoA from the cytoplasmic to the luminal side of the endoplasmic reticulum membrane, where SHH palmitoylation occurs. It is an essential factor for proper embryonic development and testicular organogenesis.

References:

[1]. John A Buglino, et al. Hhat is a palmitoylacyltransferase with specificity for N-palmitoylation of Sonic Hedgehog. J Biol Chem. 2008 Aug 8;283(32):22076-88. [Content Brief]

[2]. Z Chamoun, et al. Skinny hedgehog, an acyltransferase required for palmitoylation and activity of the hedgehog signal. Science. 2001 Sep 14;293(5537):2080-4. [Content Brief]

[3]. James J Asciolla, et al. Hedgehog Acyltransferase Promotes Uptake of Palmitoyl-CoA across the Endoplasmic Reticulum Membrane. Cell Rep. 2019 Dec 24;29(13):4608-4619.e4. [Content Brief]

[4]. Patrick Callier, et al. Loss of function mutation in the palmitoyl-transferase HHAT leads to syndromic 46,XY disorder of sex development by impeding Hedgehog protein palmitoylation and signaling. PLoS Genet. 2014 May 1;10(5):e1004340. [Content Brief]

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