CSTB Protein (AA 1-98, full length) (GST tag)

Produktnummer ABIN7479584

Produktdetails

Target: CSTB (Cystatin B (Stefin B) (CSTB))

Protein-Typ: Recombinant

Proteineigenschaft: AA 1-98, full length

Spezies: Human

Quelle: Escherichia coli (E. coli)

Aufreinigungstag / Konjugat: Dieses CSTB Protein ist gelabelt mit GST tag.

Applikation: SDS-PAGE (SDS), Western Blotting (WB), ELISA

Sequenz: MMCGAPSATQ PATAETQHIA DQVRSQLEEK ENKKFPVFKA VSFKSQVVAG TNYFIKVHVG DEDFVHLRVF QSLPHENKPL TLSNYQTNKA KHDELTYF

Reinheit: 95 %

Anwendungsinformationen

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Buffer: PBS buffer, 20mM GSH.

Lagerung: 4 °C

Antigendetails

Target: CSTB (Cystatin B (Stefin B) (CSTB))

Andere Bezeichnung: Cystatin-B (CSTB Produkte)

Synonyme: CST6 Protein, EPM1 Protein, EPM1A Protein, PME Protein, STFB Protein, ULD Protein, CSTB Protein, Cyb Protein, Epm1 Protein, Stfb Protein, MGC189005 Protein, pme Protein, cst6 Protein, epm1 Protein, stfb Protein, AA960480 Protein, ARABIDOPSIS THALIANA PHYTOCYSTATIN 6 Protein, ATCYS6 Protein, ATCYSB Protein, cystatin B Protein, LOC100008600 Protein, LOC100136235 Protein, CPI1 Protein, cystatin B Protein, cystatin B (stefin B) Protein, cystatin 14a, tandem duplicate 2 Protein, cystatin-B Protein, CSTB Protein, Cstb Protein, cstb Protein, cst14a.2 Protein, CYSB Protein, cpi1 Protein, LOC101123265 Protein

Hintergrund: Background: This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B. Defects in CSTB are the cause of progressive myoclonic epilepsy type 1 (EPM1) [MIM:254800]. EPM1 is an autosomal recessive disorder characterized by severe, stimulus-sensitive myoclonus and tonic-clonic seizures. The onset, occurring between 6 and 13 years of age, is characterized by convulsions. Myoclonus begins 1 to 5 years later. The twitchings occur predominantly in the proximal muscles of the extremities and are bilaterally symmetrical, although asynchronous. At first small, they become late in the clinical course so violent that the victim is thrown to the floor. Mental deterioration and eventually dementia develop. Synonyms: CPI-B Liver thiol proteinase inhibitor Stefin-B.

Molekulargewicht: 38.5 kD

NCBI Accession: NM_000100

UniProt: P04080

Pathways: Response to Water Deprivation