免疫原
The antiserum was produced against synthesized peptide derived from human MBTPS2. AA range:301-350
特异性
S2P Monoclonal Antibody detects endogenous levels of S2P protein.
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺（Immunogen）
The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen.
背景（Background）
This gene encodes a 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],
功能
catalytic activity: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. SREBP-2 is cleaved at the site 477-DRSRILL-|-CVLTFLCLSFNPLTSLLQWGGA-505. The residues Asn-Pro, 11 residues distal to the site of cleavage in the membrane-spanning domain, are important for cleavage by S2P endopeptidase. Replacement of either of these residues does not prevent cleavage, but there is no cleavage if both of these residues are replaced.,cofactor:Binds 1 zinc ion per subunit.,function:Intramembrane proteolysis of sterol-regulatory element-binding proteins (SREBPs) within the first transmembrane segment thereby releasing the N-terminal segment with a portion of the transmembrane segment attached. Site-2 cleavage com
蛋白名称
Membrane-bound transcription factor site-2 protease
其他名称
MBTPS2; S2P; Membrane-bound transcription factor site-2 protease; Endopeptidase S2P; Sterol regulatory element-binding proteins intramembrane protease; SREBPs intramembrane protease
Fields
>>Protein processing in endoplasmic reticulum
细胞定位
Membrane ; Multi-pass membrane protein . Cytoplasm .
组织表达
Expressed in heart, brain, placenta, lung, liver, muscle, kidney and pancreas.
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