3-dehydrosphinganine reductase

{{Short description|Protein-coding gene in the species Homo sapiens}}

{{infobox enzyme

| Name = 3-dehydrosphinganine reductase

| EC_number = 1.1.1.102

| CAS_number = 37250-36-5

| GO_code = 0047560

| image =

| width =

| caption =

}}

3-dehydrosphinganine reductase ({{EC number|1.1.1.102}}) also known as 3-ketodihydrosphingosine reductase (KDSR) or follicular variant translocation protein 1 (FVT1) is an enzyme that in humans is encoded by the KDSR gene.{{cite journal | vauthors = Rimokh R, Gadoux M, Berthéas MF, Berger F, Garoscio M, Deléage G, Germain D, Magaud JP | title = FVT-1, a novel human transcription unit affected by variant translocation t(2;18)(p11;q21) of follicular lymphoma | journal = Blood | volume = 81 | issue = 1 | pages = 136–42 | date = January 1993 | pmid = 8417785 | doi = 10.1182/blood.V81.1.136.136| doi-access = free }}{{cite journal | vauthors = Kihara A, Igarashi Y | title = FVT-1 is a mammalian 3-ketodihydrosphingosine reductase with an active site that faces the cytosolic side of the endoplasmic reticulum membrane | journal = The Journal of Biological Chemistry | volume = 279 | issue = 47 | pages = 49243–50 | date = November 2004 | pmid = 15328338 | doi = 10.1074/jbc.M405915200 | doi-access = free }}{{cite journal | vauthors = Krebs S, Medugorac I, Röther S, Strässer K, Förster M | title = A missense mutation in the 3-ketodihydrosphingosine reductase FVT1 as candidate causal mutation for bovine spinal muscular atrophy | journal = Proceedings of the National Academy of Sciences of the United States of America | volume = 104 | issue = 16 | pages = 6746–51 | date = April 2007 | pmid = 17420465 | pmc = 1868895 | doi = 10.1073/pnas.0607721104 | bibcode = 2007PNAS..104.6746K | doi-access = free }}{{cite journal | vauthors = Persson B, Kallberg Y, Bray JE, Bruford E, Dellaporta SL, Favia AD, Duarte RG, Jörnvall H, Kavanagh KL, Kedishvili N, Kisiela M, Maser E, Mindnich R, Orchard S, Penning TM, Thornton JM, Adamski J, Oppermann U | title = The SDR (short-chain dehydrogenase/reductase and related enzymes) nomenclature initiative | journal = Chemico-Biological Interactions | volume = 178 | issue = 1–3 | pages = 94–8 | date = March 2009 | pmid = 19027726 | pmc = 2896744 | doi = 10.1016/j.cbi.2008.10.040 }}{{cite web | title = Entrez Gene: FVT1 follicular lymphoma variant translocation 1| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2531}}

Function

3-dehydrosphinganine reductase catalyzes the chemical reaction:

:sphinganine + NADP⁺ $\rightleftharpoons$ 3-dehydrosphinganine + NADPH + H⁺

Thus, the two substrates of this enzyme are sphinganine and NADP⁺, whereas its 3 products are 3-dehydrosphinganine, NADPH, and H⁺.

This enzyme belongs to the family of oxidoreductases, specifically those acting on the CH-OH group of donor with NAD⁺ or NADP⁺ as acceptor. This enzyme participates in sphingolipid metabolism.

Tissue distribution

Follicular lymphoma variant translocation 1 is a secreted protein which is weakly expressed in hematopoietic tissue.

Clinical significance

FVT1 shows a high rate of transcription in some T cell malignancies and in phytohemagglutinin-stimulated lymphocytes. The proximity of FVT1 to BCL2 suggests that it may participate in the tumoral process.

References

External links

{{UCSC gene info|KDSR}}