Rabbit anti-Human MBTPS2 Polyclonal Antibody | anti-MBTPS2 antibody

MBTPS2, NT (MBTPS2, S2P, Membrane-bound transcription factor site-2 protease, Endopeptidase S2P, Sterol regulatory element-binding proteins intramembrane protease) (AP)

Cat. Num. AAA6319463

• Gene Names: MBTPS2; S2P; IFAP; KFSDX; BRESEK
• Reactivity: Human
• Applications: Immunohistochemistry, ELISA, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: MBTPS2; Polyclonal Antibody; NT (MBTPS2; S2P; Membrane-bound transcription factor site-2 protease; Endopeptidase S2P; Sterol regulatory element-binding proteins intramembrane protease) (AP); Sterol regulatory element-binding proteins intramembrane protease; anti-MBTPS2 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phosphatase (AP).

• Applicable Applications for anti-MBTPS2 antibody: Immunohistochemistry (IHC), ELISA (EIA), Western Blot (WB)
• Application Notes: IHC: 1:10-50; ELISA: 1:1,000; WB: 1:100-500; Applications are based on unconjugated antibody.
• Immunogen: MBTPS2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 14-44 amino acids from the N-terminal region of human MBTPS2.
• Conjugate: AP
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(MBTPS2 Antibody (N-term) western blot analysis in NCI-H292 cell line lysates (35ug/lane).This demonstrates the MBTPS2 antibody detected the MBTPS2 protein (arrow).)

Immunohistochemistry (IHC)

(MBTPS2 Antibody (N-term) immunohistochemistry analysis in formalin fixed and paraffin embedded human kidney tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of MBTPS2 Antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated.)

Related Product Information for anti-MBTPS2 antibody

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.

Product Categories/Family for anti-MBTPS2 antibody

Antibodies; Transcription Factors

References

Ming, A., et al. Pediatr Dermatol 26(4):427-431(2009). Oeffner, F., et al. Am. J. Hum. Genet. 84(4):459-467(2009). Lu, Y., et al. J. Lipid Res. 49(12):2582-2589(2008). Shen, J., et al. J. Biol. Chem. 279(41):43046-43051(2004). Lee, K., et al. Genes Dev. 16(4):452-466(2002).

NCBI and Uniprot Product Information

• NCBI GI #: 7706693
• NCBI GeneID: 51360
• NCBI Accession #: NP_056968.1
• NCBI GenBank Nucleotide #: NM_015884.3
• UniProt Accession #: O43462; Q9UM70; Q9UMD3
• Molecular Weight: 57,444 Da
• NCBI Official Full Name: membrane-bound transcription factor site-2 protease
• NCBI Official Synonym Full Names: membrane-bound transcription factor peptidase, site 2
• NCBI Official Symbol: MBTPS2
• NCBI Official Synonym Symbols: S2P; IFAP; KFSDX; BRESEK
• NCBI Protein Information: membrane-bound transcription factor site-2 protease; endopeptidase S2P; SREBPs intramembrane protease; membrane-bound transcription factor protease, site 2; sterol regulatory element-binding proteins intramembrane protease
• UniProt Protein Name: Membrane-bound transcription factor site-2 protease
• Protein Family: Membrane-bound transcription factor site-2 protease
• UniProt Gene Name: MBTPS2
• UniProt Synonym Gene Names: S2P; SREBPs intramembrane protease
• UniProt Entry Name: MBTP2_HUMAN

NCBI Description

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]

Uniprot Description

MBTPS2: 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. Expressed in heart, brain, placenta, lung, liver, muscle, kidney and pancreas. Belongs to the peptidase M50A family.

Protein type: Membrane protein, integral; Membrane protein, multi-pass; Protease; EC 3.4.24.85

Chromosomal Location of Human Ortholog: Xp22.12-p22.11

Cellular Component: Golgi membrane; cytoplasm; integral to membrane

Molecular Function: metalloendopeptidase activity; metal ion binding

Biological Process: cholesterol metabolic process; cellular protein metabolic process; unfolded protein response, activation of signaling protein activity; membrane protein intracellular domain proteolysis; unfolded protein response; positive regulation of transcription factor activity

Disease: Ifap Syndrome With Or Without Bresheck Syndrome; Palmoplantar Keratoderma, Mutilating, With Periorificial Keratotic Plaques, X-linked; Keratosis Follicularis Spinulosa Decalvans, X-linked