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Rabbit Anti-Site 2 protease  antibody (bs-19356R)  
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產品編號 bs-19356R
英文名稱 Rabbit Anti-Site 2 protease  antibody
中文名稱 S2P內肽酶抗體
別    名 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.  
研究領域 細胞生物  免疫學  泛素  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse,Rat (predicted: Human,Sheep,Cow,Horse)
產品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 57kDa
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Site 2 protease: 1-100/519 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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



產品圖片
Sample: Heart (Mouse) Lysate at 40 ug Primary: Anti-Site 2 protease? (bs-19356R) 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? (bs-19356R) 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 (bs-19356R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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