SHPK phosphorylates Sedo to Sedo7P

Stable Identifier
R-HSA-8959719
Type
Reaction [transition]
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
22682222 The sedoheptulose kinase CARKL directs macrophage polarization through control of glucose metabolism

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

Cell Metab. 2012
18775706 Characterization of mammalian sedoheptulokinase and mechanism of formation of erythritol in sedoheptulokinase deficiency

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

FEBS Lett. 2008
18186520 Sedoheptulokinase deficiency due to a 57-kb deletion in cystinosis patients causes urinary accumulation of sedoheptulose: elucidation of the CARKL gene

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

Hum. Mutat. 2008
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
sedoheptulokinase activity of SHPK [cytosol]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
Authored
Reviewed
Created
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