Membrane-bound transcription factor site-2 protease

Membrane-bound transcription factor site-2 protease, also known as S2P endopeptidase or site-2 protease (S2P), is an enzyme (EC 3.4.24.85) encoded by the MBTPS2 gene which liberates the N-terminal fragment of sterol regulatory element-binding protein (SREBP) transcription factors from membranes.^[1][2] S2P cleaves the transmembrane domain of SREBP, making it a member of the class of intramembrane proteases.^[3]

membrane-bound transcription factor peptidase, site 2
Identifiers
Symbol	MBTPS2
Alt. symbols	S2P
NCBI gene	51360
HGNC	15455
OMIM	300294
RefSeq	NM_015884
UniProt	O43462
Other data
EC number	3.4.24.85
Locus	Chr. X p22.1-p22.2
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S2P endopeptidase
Identifiers
EC no.	3.4.24.85
CAS no.	752251-31-3
Databases
IntEnz	IntEnz view
BRENDA	BRENDA entry
ExPASy	NiceZyme view
KEGG	KEGG entry
MetaCyc	metabolic pathway
PRIAM	profile
PDB structures	RCSB PDB PDBe PDBsum
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S2P catalyses the following chemical reaction

Cleaves several transcription factors that are type-2 transmembrane proteins within membrane-spanning domains. Known substrates include sterol regulatory element-binding protein (SREBP)-1, SREBP-2 and forms of the transcriptional activator ATF6.

This enzyme belongs to the peptidase family M50.

Function

This gene encodes an intramembrane zinc metalloprotease, which is essential in development. This protease functions in the signal protein activation involved in sterol control of transcription and the ER stress response. Mutations in this gene have been associated with ichthyosis follicularis with atrichia and photophobia (IFAP syndrome); IFAP syndrome has been quantitatively linked to a reduction in cholesterol homeostasis and ER stress response.[provided by RefSeq, Aug 2009].

See also

• Membrane-bound transcription factor site-1 protease