Acetylation of proteins is a post-translational modification mediated by specific acetyltransferases, using the donor acetyl CoA. SLC33A1/AT1 is a putative 11 TM transporter present on the endoplasmic reticulum, expressed in all tissues, but particularly abundant in the pancreas [2], which imports cytosolic acetyl CoA into these intracellular organelles.

In heterologous expression studies, acetyl CoA transport through AT1 was inhibited by coenzyme A, but not acetic acid, ATP or UDP-galactose [1]. A loss-of-function mutation in ACATN1/SLC33A1 has been associated with spastic paraplegia (SPG42, [3]), although this observation could not be replicated in a subsequent study [4].

* Key recommended reading is highlighted with an asterisk

* Hirabayashi Y, Kanamori A, Nomura KH, Nomura K. (2004) The acetyl-CoA transporter family SLC33. Pflugers Arch., 447 (5): 760-2. [PMID:12739170]

* Hirabayashi Y, Nomura KH, Nomura K. (2013) The acetyl-CoA transporter family SLC33. Mol. Aspects Med., 34 (2-3): 586-9. [PMID:23506891]

1. Jonas MC, Pehar M, Puglielli L. (2010) AT-1 is the ER membrane acetyl-CoA transporter and is essential for cell viability. J. Cell. Sci., 123 (Pt 19): 3378-88. [PMID:20826464]

2. Kanamori A, Nakayama J, Fukuda MN, Stallcup WB, Sasaki K, Fukuda M, Hirabayashi Y. (1997) Expression cloning and characterization of a cDNA encoding a novel membrane protein required for the formation of O-acetylated ganglioside: a putative acetyl-CoA transporter. Proc. Natl. Acad. Sci. U.S.A., 94 (7): 2897-902. [PMID:9096318]

3. Lin P, Li J, Liu Q, Mao F, Li J, Qiu R, Hu H, Song Y, Yang Y, Gao G et al.. (2008) A missense mutation in SLC33A1, which encodes the acetyl-CoA transporter, causes autosomal-dominant spastic paraplegia (SPG42). Am. J. Hum. Genet., 83 (6): 752-9. [PMID:19061983]

4. Schlipf NA, Beetz C, Schüle R, Stevanin G, Erichsen AK, Forlani S, Zaros C, Karle K, Klebe S, Klimpe S et al.. (2010) A total of 220 patients with autosomal dominant spastic paraplegia do not display mutations in the SLC33A1 gene (SPG42). Eur. J. Hum. Genet., 18 (9): 1065-7. [PMID:20461110]