Membrane-bound transcription factor site-2 protease (IPR001193)

Short name: MBTPS2

Overlapping homologous superfamilies


Family relationships



This entry represents the membrane-bound transcription factor site-2 protease (MBTPS2, also known as S2P)[PMID: 19361614].

MBTPS2 is a membrane-embedded zinc metalloprotease that activates signaling proteins involved in sterol control of transcription and ER stress response [PMID: 19361614]. It cleaves several transcription factors that are type-2 transmembrane proteins within membrane-spanning domains. Its known substrates include sterol regulatory element-binding protein (SREBP) -1, SREBP-2 and forms of the transcriptional activator ATF6 [PMID: 9659902, PMID: 10419520].

Mutations in the MBTPS2 gene cause IFAP syndrome with or without BRESHECK syndrome (IFAPS) and Keratosis follicularis spinulosa decalvans X-linked (KFSDX) [PMID: 19361614, PMID: 20672378].

GO terms

Biological Process

GO:0006508 proteolysis

Molecular Function

GO:0004222 metalloendopeptidase activity

Cellular Component

GO:0016020 membrane

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.