Mouse anti-Human MBTPS2 Monoclonal Antibody | anti-MBTPS2 antibody

MBTPS2 (S2P, Membrane-bound Transcription Factor Site-2 Protease, Endopeptidase S2P, Sterol Regulatory Element-binding Proteins Intramembrane Protease, FLJ32174) (FITC)

Cat. Num. AAA6148157

• Gene Names: MBTPS2; S2P; IFAP; KFSDX; BRESEK
• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: MBTPS2; Monoclonal Antibody; MBTPS2 (S2P; Membrane-bound Transcription Factor Site-2 Protease; Endopeptidase S2P; Sterol Regulatory Element-binding Proteins Intramembrane Protease; FLJ32174) (FITC); anti-MBTPS2 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG1,k
• Clone Number: 1A3
• Specificity: Recognizes human MBTPS2.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein isothiocyanate (FITC).

• Applicable Applications for anti-MBTPS2 antibody: ELISA (EIA), Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Partial recombinant corresponding to aa312-419 from human MBTPS2 (NP_056968) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: ASTLQQLSFPVRAYKRLDGSTECCNNHSLTDVCFSYRNNFNKRLHTCLPARKAVEATQVCRTNKDCKKSSSSSFCIIPSLETHTRLIKVKHPPQIDMLYVGHPLHLH
• Conjugate: FITC
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (37.88kD).)

Testing Data

(Detection limit for recombinant GST tagged MBTPS2 is 1ng/ml as a capture antibody.)

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

NCBI and Uniprot Product Information

• NCBI GI #: 7706693
• NCBI GeneID: 51360
• NCBI Accession #: NP_056968.1
• NCBI GenBank Nucleotide #: NM_015884
• 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