Recombinant human Cystatin-B protein

Relevance: 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
Product Info: GST tagged
Source: E.coli derived
Purity: 95%
Image:
Tested applications: SDS-PAGE, ELISA, Western blot
Storage Buffer: PBS buffer,20mM GSH.
Storage: Store at -20?, for extended storage, conserve at -20? or -80?.
Notes: Repeated freezing and thawing is not recommended. Store working aliquots at 4? for up to one week.
AA sequence: MMCGAPSATQPATAETQHIADQVRSQLEEKENKKFPVFKAVSFKSQVVAGTNYFIKVHVGDEDFVHLRVFQSLPHENKPLTLSNYQTNKAKHDELTYF-
Referrences: 1] "Amino acid sequence of the intracellular cysteine proteinase inhibitor cystatin B from human liver." Ritonja A., Machleidt W., Barrett A.J. Biochem. Biophys. Res. Commun. 131:1187-1192(1985) [PubMed: 3902020] [Abstract] Cited for: PROTEIN SEQUENCE. [2] "Mutations in the gene encoding cystatin B in progressive myoclonus epilepsy (EPM1)." Pennacchio L.A., Lehesjoki A.-E., Stone N.E., Willour V.L., Virteneva K., Miao J., D'Amato E., Ramirez L., Faham J., Koskiniemi M., Warringtion J.A., Norio R., la Chapelle A., Cox D.R., Myers R.M. Science 271:1731-1734(1996) [PubMed: 8596935] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA]. [3] Bhat K.S. Submitted (MAY-1993) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [MRNA].
Alias: CPI-B,Liver thiol proteinase inhibitor,Stefin-B.

Информация для заказа

Область использования: Рекомбинантные белки Cusabio	Производство:	Cusabio
	Метод:
	Объем:	500 мкг
	Кат. номер:	CSB-RP002244h
Цена (с НДС 20%):	по запросу	В корзину
Наименование: Recombinant human Cystatin-B protein. Примечание: дополнительная информация (на английском языке).