SLC33A1 transports Ac-CoA from cytosol to Golgi lumen

Stable Identifier
Reaction [transition]
Homo sapiens
Locations in the PathwayBrowser
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

The human gene SLC33A1 encodes acetyl-CoA transporter AT1 (Kanamori et al. 1997). Acetyl-CoA is transported to the lumen of the Golgi apparatus, where it serves as the substrate of acetyltransferases that O-acetylates sialyl residues of gangliosides and glycoproteins. Defects in SLC33A1 are the cause of spastic paraplegia autosomal dominant type 42 (SPG42) which is a neurodegenerative disorder (Lin et al. 2008).

Literature References
PubMed ID Title Journal Year
9096318 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

Fukuda, M, Kanamori, A, Sasaki, K, Stallcup, WB, Hirabayashi, Y, Nakayama, J, Fukuda, MN

Proc Natl Acad Sci U S A 1997
19061983 A missense mutation in SLC33A1, which encodes the acetyl-CoA transporter, causes autosomal-dominant spastic paraplegia (SPG42)

Qiu, R, Yang, W, Yan, C, Gao, G, Liu, Q, Hu, H, Gong, Y, Mao, F, Lin, P, Shao, C, Li, J, Li, J, Yang, Y, Song, Y

Am J Hum Genet 2008
Catalyst Activity

acetyl-CoA transmembrane transporter activity of SLC33A1 [Golgi membrane]

Orthologous Events
Cite Us!