CSTB-antilichaam - middengebied: FITC (ARP45748_P050-FITC)

Cystatin B (stefin B)

PME, ULD, CST6, EPM1, STFB, CPI-B, EPM1A

1476

P04080

NP_000091

Human, Mouse, Rat, Dog, Horse, Pig, Rabbit

The immunogen is a synthetic peptide directed towards the middle region of human CSTB

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) .

UBC; SARNP; DPP7; BAG3; CFTR; ECT2; SEC24A; NUDC; MAT2A; FLNB; DCUN1D1; CUL3; RAD21; SPRY2; CTSL; CTSD; CTSH; CTSB; CST3; VHL

FITC: Fluorescein Isothiocyanate

Polyclonal Antibody

IHC, WB

Affinity Purified

0.5 mg/ml

Dog: 92%; Horse: 85%; Human: 100%; Mouse: 83%; Pig: 79%; Rabbit: 77%; Rat: 79%

Liquid. Purified antibody supplied in 1x PBS buffer.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

11kDa

Kenig, M., (2006) Proteins 62 (4), 918-927

Wet Ice

98

CSTB

CSTB

Rabbit

Cystatin-B

NM_000100

AGTNYFIKVHVGDEDFVHLRVFQSLPHENKPLTLSNYQTNKAKHDELTYF