-
Gene info
CSTB is 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 CSTB gene are responsible for the primary defects in patients with progressive myoclonic epilepsy 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.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).
-
Gene names
CSTB, CST6, EPM1, PME, STFB, ULD, EPM1A
-
Antibodie s label
unlabelled serum
-
Purity
Antibody is purified by peptide affinity chromatography method.
-
Ab raised in
Rabbit
-
Type
Polyclonal
-
Antigen
Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human CSTB.
-
Immuno application
ELISA, Western Blot, immuno-histo-chemistry
-
Antibody advice
CSTB antibody can be used for detection of CSTB by ELISA at 1:12500. CSTB antibody can be used for detection of CSTB by western blot at 0.25 ug/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.
-
Suspention pH azide
Antibody is lyophilized in PBS buffer with 2% sucrose. Add 50 μL of distilled water. Final antibody concentration is 1 mg/mL.
-
Keep at
For short periods of storage (days) store at 4 grades C. For longer periods of storage, store CSTB antibody at -20 grades C. As with any antibody avoid repeat freeze-thaw cycles.
-
Works with
Human
-
Omim nr
4503117
-
Ncbi nr
NP_000091
-
KDa
11 kDa
-
Vial with antibody
Lyophilized
-
Quantity per ml
1 mg/ml
-
NCBI GENE nr
1476
-
GENE symbol
CSTB
-
Antigene name
cystatin B (stefin B)
-
Source
Homo sapiens
-
Protein nr
P04080
-
-
-
-
Description
This antibody needs to be stored at + 4°C in a fridge short term in a concentrated dilution. Freeze thaw will destroy a percentage in every cycle and should be avoided. Antibody for research use.
-
Properties
If you buy Antibodies supplied by proscience they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
-
French translation
anticorps
