Description
Antibody Name: | MBTPS2 Antibody |
Antibody SKU: | PACO04534 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, IF |
Recommended Dilutions: | IF:1:200-1:1000 |
Species Reactivity: | Human, Mouse, Rat |
Immunogen: | synthesized peptide derived from the Internal region of human S2P. |
Form: | Liquid |
Storage Buffer: | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
Purification Method: | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Synonyms: | MBTPS2; S2P; Membrane-bound transcription factor site-2 protease; Endopeptidase S2P; Sterol regulatory element-binding proteins intramembrane protease; SREBPs intramembrane protease |
UniProt Protein Function: | 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, multi-pass; Protease; EC 3.4.24.85; Membrane protein, integralChromosomal Location of Human Ortholog: Xp22.12-p22.11Cellular Component: cytoplasm; Golgi membraneMolecular Function: metallopeptidase activityBiological Process: membrane protein intracellular domain proteolysis; positive regulation of transcription factor activityDisease: Ifap Syndrome With Or Without Bresheck Syndrome; Keratosis Follicularis Spinulosa Decalvans, X-linked; Palmoplantar Keratoderma, Mutilating, With Periorificial Keratotic Plaques, X-linked |
UniProt Protein Details: | |
NCBI Summary: | 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 Code: | O43462 |
NCBI GenInfo Identifier: | 6016601 |
NCBI Gene ID: | 51360 |
NCBI Accession: | O43462.1 |
UniProt Secondary Accession: | O43462,Q9UM70, Q9UMD3 |
UniProt Related Accession: | O43462 |
Molecular Weight: | 57,444 Da |
NCBI Full Name: | Membrane-bound transcription factor site-2 protease |
NCBI Synonym Full Names: | membrane bound transcription factor peptidase, site 2 |
NCBI Official Symbol: | MBTPS2 |
NCBI Official Synonym Symbols: | S2P; IFAP; KFSD; KFSDX; OLMSX; BRESEK |
NCBI Protein Information: | membrane-bound transcription factor site-2 protease |
UniProt Protein Name: | Membrane-bound transcription factor site-2 protease |
UniProt Synonym Protein Names: | Endopeptidase S2P; Sterol regulatory element-binding proteins intramembrane protease; SREBPs intramembrane protease |
Protein Family: | Membrane-bound transcription factor site-2 protease |
UniProt Gene Name: | MBTPS2 |
UniProt Entry Name: | MBTP2_HUMAN |