SHPK phosphorylates Sedo to Sedo7P

Stable Identifier
R-HSA-8959719
Type
Reaction
Species
Homo sapiens
Compartment
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Cytosolic sedoheptulokinase (SHPK aka CARKL) catalyses an orphan reaction in the pentose phosphate pathway and is a novel regulator of glycolytic energy flux which is critical for macrophage activation (Haschemi et al. 2012). The most common mutation in the nephropathic cystinosis (CTNS) gene is a homozygous 57-kb deletion that also includes the adjacent gene SHPK. In nephropathic cystinosis patients, defects in SHPK can cause urinary accumulation of sedoheptulose and erythritol (Wamelink et al. 2008, Kardon et al. 2008).

Literature References
PubMed ID Title Journal Year
18775706 Characterization of mammalian sedoheptulokinase and mechanism of formation of erythritol in sedoheptulokinase deficiency

Stroobant, V, Kardon, T, Schaftingen, EV, Veiga-da-Cunha, M

FEBS Lett. 2008
22682222 The sedoheptulose kinase CARKL directs macrophage polarization through control of glucose metabolism

Park, J, Bilban, M, Schmid, JA, Burant, CF, Esterbauer, H, Pospisilik, JA, Brizuela, L, Haschemi, A, Kosma, P, Lubec, G, Jandl, C, Wagner, O, Starkl, P, Gille, L, Knapp, B, Evans, CR, Haas, R, Amir, S, Otterbein, LE

Cell Metab. 2012
18186520 Sedoheptulokinase deficiency due to a 57-kb deletion in cystinosis patients causes urinary accumulation of sedoheptulose: elucidation of the CARKL gene

Jansen, EE, Wevers, RA, Engelke, U, Wamelink, MM, Zijlstra, FS, Jakobs, C, Struys, EA, Blom, HJ, Levtchenko, EN

Hum. Mutat. 2008
Participants
Participates
Catalyst Activity

sedoheptulokinase activity of SHPK [cytosol]

Orthologous Events
Cross References
Rhea
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