Product Name	Site 2 protease
Chinese Name	S2P内肽酶抗体
Alias	BRESEK; Endopeptidase S2P; IFAP; KFSDX; MBTP2_HUMAN; MBTPS2; Membrane bound transcription factor site 2 protease; membrane-bound transcription factor peptidase, site 2; membrane-bound transcription factor protease, site 2; Membrane-bound transcription factor site-2 protease; S2P; S2P endopeptidase; Site 2 protease; SREBPs intramembrane protease; Sterol regulatory element-binding proteins intramembrane protease.
Research Area	Cell biology  immunology  Ubiquitin
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Mouse, Rat,  (predicted: Human, Cow, Horse, Sheep, )
Applications	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	57kDa
Cellular localization	cytoplasmic The cell membrane
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human Site 2 protease: 1-100/519
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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] 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 comes after site-1 cleavage which takes place in the lumenal loop. Subcellular Location: Membrane. Cytoplasm. Tissue Specificity: Expressed in heart, brain, placenta, lung, liver, muscle, kidney and pancreas. DISEASE: Defects in MBTPS2 are the cause of ichthyosis follicularis-atrichia-photophobia syndrome (IFAPS) [MIM:308205]. A syndrome characterized by a peculiar triad of follicular ichthyosis, total or subtotal atrichia, and photophobia of varying degree. Histopathologically, the epidermal granular layer is generally well-preserved or thickened at the infundibulum. Hair follicles are poorly developed and tend to be surrounded by an inflammatory infiltrate. A subgroup of patients is described with lamellar rather than follicular ichthyosis. Non-consistent features may include growth and psychomotor retardation, aganglionic megacolon, seizures and nail dystrophy. Defects in MBTPS2 are a cause of keratosis follicularis spinulosa decalvans X-linked (KFSDX) [MIM:308800]. A rare disorder affecting the skin and the eye. Affected men show thickening of the skin of the neck, ears, and extremities, especially the palms and soles, loss of eyebrows, eyelashes and beard, thickening of the eyelids with blepharitis and ectropion, and corneal degeneration. Similarity: Belongs to the peptidase M50A family. SWISS: O43462 Gene ID: 51360 Database links: Entrez Gene: 51360 Human Entrez Gene: 270669 Mouse Entrez Gene: 302705 Rat Omim: 300294 Human SwissProt: O43462 Human SwissProt: Q8CHX6 Mouse Unigene: 443490 Human Unigene: 37577 Mouse Unigene: 212224 Rat
Product Picture	Sample: Heart (Mouse) Lysate at 40 ug Primary: Anti-Site 2 protease  (SL19356R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 57 kD Observed band size: 57 kD Sample: H9C2(Rat) Cell Lysate at 30 ug Primary: Anti-Site 2 protease  (SL19356R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 57 kD Observed band size: 57 kD Paraformaldehyde-fixed, paraffin embedded (Mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Site 2 protease) Polyclonal Antibody, Unconjugated (SL19356R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.

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