For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against CSTB. It has beenselected for its ability to recognize CSTB in immunohistochemical staining andwestern blotting.

Affinity Chromatography

Liquid

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of anti-CSTB antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Knock-Out Validation Antibody

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA)

Western blotting: 0.2-2ug/mL;1:250-2500
Immunohistochemistry: 5-20ug/mL;1:25-100
Immunocytochemistry: 5-20ug/mL;1:25-100
Optimal working dilutions must be determined by end user.

Western Blot: Sample: Recombinant protein.

DAB staining on fromalin fixed paraffin-embedded Liver tissue)

FITC staining on IHC-P Simple: Mcf7 cells)

Knockout Validation: Lane 1: Wild-type HepG2 cell lysate;;Lane 2: CSTB knockout HepG2 cell lysate;;Predicted MW: 11kDa ;Observed MW: 14kDa;Primary Ab: 2ug/ml Rabbit Anti-Human CSTB Antibody;Second Ab: 0.2ug/mL?HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody;(#MBS2086047)

11,140 Da

cystatin B

cystatin-B

Cystatin-B

CST6; STFB??[Similar Products]

The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and kininogens. This gene encodes a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in this gene are responsible for the primary defects in patients with progressive myoclonic epilepsy (EPM1). One type of mutation responsible for EPM1 is the expansion in the promoter region of this gene of a CCCCGCCCCGCG repeat from 2-3 copies to 30-78 copies. [provided by RefSeq, Jul 2016]

CSTB: 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). 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. Belongs to the cystatin family.

Protein type: Inhibitor

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: cytoplasm; cytosol; extracellular exosome; extracellular region; extracellular space; ficolin-1-rich granule lumen; nucleolus; secretory granule lumen; tertiary granule lumen

Molecular Function: cysteine-type endopeptidase inhibitor activity; endopeptidase inhibitor activity; protease binding; RNA binding

Biological Process: ***** locomotory behavior; negative regulation of endopeptidase activity; negative regulation of peptidase activity; negative regulation of proteolysis; neutrophil degranulation

Disease: Myoclonic Epilepsy Of Unverricht And Lundborg

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