Reactome | SLC35B2,3 exchange cytosolic PAPS with Golgi PAP

SLC35B2,3 exchange cytosolic PAPS with Golgi PAP

Stable Identifier

R-HSA-741449

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane, Golgi lumen, cytosol

ReviewStatus

5/5

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SLC35B2,3 exchange cytosolic PAPS with Golgi PAP

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The human gene SLC35B2 encodes the adenosine 3'-phospho 5'-phosphosulfate transporter 1 (PAPST1) (Ozeran et al. 1996, Kamiyama et al. 2003). In human tissues, PAPST1 is highly expressed in the placenta and pancreas and present at lower levels in the colon and heart. The human gene SLC35B3 encodes a human PAPS transporter gene that is closely related to PAPST1. Called PAPST2, it is predominantly expressed in the colon (Kamiyama et al. 2006). Both proteins can transport PAPS from the cytosol to the Golgi lumen, simultaneously antiporting PAP back to cytosol. Mutations in SLC35B2 can lead to hypomyelinating leukodystrophy (HLD26, MIM:620269; Guasto et al., 2022).

Literature References

PubMed ID	Title	Journal	Year
35325049	Biallelic variants in SLC35B2 cause a novel chondrodysplasia with hypomyelinating leukodystrophy Guasto, A, Dubail, J, Aguilera-Albesa, S, Paganini, C, Vanhulle, C, Haouari, W, Gorría-Redondo, N, Aznal-Sainz, E, Boddaert, N, Planas-Serra, L, Schlüter, A, Vélez-Santamaría, V, Verdura, E, Bruneel, A, Rossi, A, Huber, C, Pujol, A, Cormier-Daire, V	Brain	2022
8619989	Identification and partial purification of PAPS translocase Ozeran, JD, Westley, J, Schwartz, NB	Biochemistry	1996
12716889	Molecular cloning and identification of 3'-phosphoadenosine 5'-phosphosulfate transporter Kamiyama, S, Suda, T, Ueda, R, Suzuki, M, Okubo, R, Kikuchi, N, Chiba, Y, Goto, S, Toyoda, H, Saigo, K, Watanabe, M, Narimatsu, H, Jigami, Y, Nishihara, S	J Biol Chem	2003
16492677	Molecular cloning and characterization of a novel 3'-phosphoadenosine 5'-phosphosulfate transporter, PAPST2 Kamiyama, S, Sasaki, N, Goda, E, Ui-Tei, K, Saigo, K, Narimatsu, H, Jigami, Y, Kannagi, R, Irimura, T, Nishihara, S	J Biol Chem	2006