SLC33A1 transports Ac-CoA from cytosol to Golgi lumen

Stable Identifier
R-HSA-727759
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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
19061983 A missense mutation in SLC33A1, which encodes the acetyl-CoA transporter, causes autosomal-dominant spastic paraplegia (SPG42)

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

Am J Hum Genet 2008
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

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

Proc Natl Acad Sci U S A 1997
Participants
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Catalyst Activity
Catalyst Activity
Title
acetyl-CoA transmembrane transporter activity of SLC33A1 [Golgi membrane]
Physical Entity
Activity
Orthologous Events
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