KDSR reduces 3-ketosphingoid

Stable Identifier
R-HSA-428123
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
3-ketosphingoid + NADPH + H+ => sphingoid + NADP+
ReviewStatus
5/5
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KDSR (3-ketodihydrosphingosine reductase) enzyme associated with the cytosolic face of the endoplasmic reticulum membrane catalyzes the reduction of 3-ketosphingoid by NADPH to form sphingoid (Kihara and Igarashi 2004). Defects in KDSR or missing activity causes Erythrokeratodermia (EKVP4, MIM:617526), associated with sphingolipid dysregulation (Boyden et al., 2017). While there is no data on substrates other than 3-ketosphinganine, it is probable that KDSR has activity toward 3-ketosphingoids with other chain lengths than C16.
Literature References
PubMed ID Title Journal Year
15328338 FVT-1 is a mammalian 3-ketodihydrosphingosine reductase with an active site that faces the cytosolic side of the endoplasmic reticulum membrane

Igarashi, Y, Kihara, A

J Biol Chem 2004
28575652 Mutations in KDSR Cause Recessive Progressive Symmetric Erythrokeratoderma

Diaz, LA, Craiglow, BG, Boyden, LM, Choate, KA, Baserga, SJ, Rosman, IS, Vincent, NG, Lifton, RP, Paller, AS, Goldsmith, LA, Hu, R, Bayliss, SJ, Lucky, AW, Zhou, J

Am J Hum Genet 2017
Participants
Participates
Catalyst Activity

3-dehydrosphinganine reductase activity of KDSR [endoplasmic reticulum membrane]

Orthologous Events
Cross References
Rhea
Authored
Reviewed
Created
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