SEPHS2 (human) — review notes

UniProt: Q99611 (SPS2_HUMAN). Gene: SEPHS2 (synonym SPS2). 448 aa. HGNC:19686.

Identity and core function

SEPHS2 = Selenide, water dikinase 2 (EC 2.7.9.3), also "Selenium donor protein 2" /
"Selenophosphate synthase 2". It is the catalytically active human selenophosphate
synthetase.

The reaction product, monoselenophosphate, is the activated selenium donor used
downstream by SEPSECS to convert O-phosphoseryl-tRNA(Sec) to selenocysteyl-tRNA(Sec),
supplying Sec for all selenoproteins.

SEPHS2 is itself a selenoprotein (autoregulation)

UniProt FT NON_STD 60 = Selenocysteine; ACT_SITE 60; KW "Selenocysteine", "Selenium".
The catalytic residue is a Sec (U) at position 60. Thus SEPHS2's own synthesis depends
on the selenocysteine biosynthesis pathway it feeds — an autoregulatory feature
PMID:8986768.
MUTAGEN U60->C "Does not affect the selenophosphate synthase activity"
(ECO:0000269|PubMed:15534230), so the Cys mutant is catalytically competent — this is why
the recombinant Sps2Cys form was used for the E. coli complementation assay.

Human paralog SEPHS1 (SPS1, P49903) does NOT contain Sec (has Thr/Arg at the homologous
position) and is proposed to act in a selenium salvage/recycling role rather than de novo
selenophosphate synthesis; its exact enzymatic function is debated. SEPHS2 is the
Sec-containing, catalytically active enzyme for de novo selenophosphate synthesis.

Complex membership / interactions

UniProt SUBUNIT: "Homodimer (By similarity). Component of a complex composed of SEPHS1,
SEPHS2, SEPSECS and TRNAU1AP" (PubMed:16508009, PubMed:28414460).
[file:human/SEPHS2/SEPHS2-uniprot.txt]

Localization

PTMs / regulation (context, not core function)

Family / domain

SIMILARITY: "Belongs to the selenophosphate synthase 1 family. Class I subfamily."
PurM-like fold (Pfam AIRS / AIRS_C; CDD SelD; TIGR selD; PANTHER PTHR10256:SF1 "SELENIDE,
WATER DIKINASE 2"). ATP- and Mg2+-binding residues annotated by similarity to P49903;
ATP is "ligand shared between dimeric partners" (consistent with the functional
homodimer). [file:human/SEPHS2/SEPHS2-uniprot.txt]

Curation summary of existing annotations

Core:
- MF GO:0004756 selenide, water dikinase activity (enables) — ACCEPT (IBA/ISS); the two
literature-supported instances (Reactome TAS R-HSA-8959510; NAS PMID:8986768) ACCEPT;
the redundant IEA also ACCEPT.
- BP GO:0016260 L-selenocysteine biosynthetic process (involved_in) — ACCEPT (IMP
PMID:15534230 is the strongest); IBA/IEA/TAS/NAS instances ACCEPT.
- CC cytoplasm/cytosol — ACCEPT (IDA PMID:28414460 strongest; IBA is_active_in and
IEA/TAS ACCEPT).
- MF GO:0005524 ATP binding (IEA, InterPro) — ACCEPT; ATP is a genuine substrate/ligand
(FT BINDING; Rhea reaction), directly part of catalysis.

Non-core:
- MF GO:0005515 protein binding (IPI PMID:33961781, with SEPHS1) — MARK_AS_OVER_ANNOTATED
(uninformative bare protein binding; per policy never REMOVE an IPI). Real interaction,
but no specific MF conveyed.

No REMOVE actions: all annotations are biologically consistent with SEPHS2 as the
Sec-containing selenophosphate synthetase.

GO term id/label checks (EBI OLS4, 2026-07)