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 comes after site-1 cleavage which takes place in the lumenal loop.Rawson R.B., Mol. Cell 1:47-57(1997).Ye J., Proc. Natl. Acad. Sci. U.S.A. 97:5123-5128(2000).
Host
Rabbit
Immunogen
Synthesized peptide derived from internal of Human MBTPS2.
Involvement In Disease
IFAP syndrome with or without BRESHECK syndrome (IFAPS); Olmsted syndrome, X-linked (OLMSX); Keratosis follicularis spinulosa decalvans X-linked (KFSDX)
Raised In
Rabbit
Reactivity
Human, Mouse
Regulatory
RUO
Relevance
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 comes after site-1 cleavage which takes place in the lumenal loop.
Rawson R.B., Mol. Cell 1:47-57(1997). Ye J., Proc. Natl. Acad. Sci. U.S.A. 97:5123-5128(2000).
Species
Homo Sapiens (Human)
Specificity
The antibody detects endogenous levels of total MBTPS2 protein.
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 comes after site-1 cleavage which takes place in the lumenal loop.
Pathway
Protein processing in endoplasmic reticulum
Protein Families
Peptidase M50A family
Tissue Specificity
Expressed in heart, brain, placenta, lung, liver, muscle, kidney and pancreas.
Buffer
Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Form
Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Format
liquid
Purification
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Purity
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.