Mouse Cystatin B Monoclonal Antibody | anti-CSTB antibody

Mouse Anti Human Cystatin B

Cat. Num. AAA140241

• Gene Names: CSTB; PME; ULD; CST6; EPM1; STFB; EPM1A
• Applications: ELISA, Western Blot
• Purity: CSTB antibody was purified from mouse ascitic fluids by protein-G affinity chromatography.
• Synonyms: Cystatin B; Monoclonal Antibody; Mouse Anti Human Cystatin B; CSTB Antibody; Mouse Anti Human; Cystatin-B; Stefin-B; Liver thiol proteinase inhibitor; CPI-B; CSTB; CST6; EPM1; PME; STFB; anti-CSTB antibody

• Host: Mouse
• Clonality: Monoclonal
• Isotype: IgG2b heavy chain and kappa light chain
• Clone Number: P2F1AT
• Purity/Purification: CSTB antibody was purified from mouse ascitic fluids by protein-G affinity chromatography.
• Form/Format: 1mg/ml containing PBS, pH-7.4, & 0.1% Sodium Azide.
• Sequence Length: 98

• Applicable Applications for anti-CSTB antibody: ELISA (EIA), Western Blot (WB)
• Application Notes: CSTB antibody has been tested by ELISA and Western blot analysis to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results. Recommended dilution range for Western blot analysis is 1:1,000~ 2,000. Recommended starting dilution is 1:1,000.
• Immunogen: Anti-human CSTB mAb is derived from hybridization of mouse F0 myeloma cells with spleen cells from BALB/c mice immunized with recombinant human CSTB amino acids 1-98 purified from E Coli.
• Preparation and Storage: For periods up to 1 month store at 4 degree C, for longer periods of time, store at -20 degree C. Prevent freeze thaw cycles.; 12 months at -20 degree C. 1 month at 4 degree C.

Related Product Information for anti-CSTB antibody

Introduction: Type 1 cystatins are also called stefins which function as intracellular thiol protease inhibitors. Cystatin-B protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. CSTB protein protects proteases leakage from lysosomes. Mutations in Stefin-B gene cause primary defects in patients with progressive myoclonic epilepsy (EPM1), a degenerative disease of the central nervous system. CSTB is overexpressed & elevated in the serum of HCC patients. Cystatin-B in vivo has a polymeric structure which is sensitive to the redox environment. Cystatin-B inhibits bone resorption by down-regulating intracellular cathepsin K activity despite increased osteoclast survival. Protein and mRNA levels of stefin B are significantly lower in atypical benign meningiomas. Stefins-A & Stefin-B which belong to the type-1 Cystatins, are up-regulated in lung tumours and thus able to counteract harmful tumour-associated proteolytic activity. Human stefin-A & Stef

Product Categories/Family for anti-CSTB antibody

Monoclonal/Polyclonal Antibodies; Monoclonal Antibodies

NCBI and Uniprot Product Information

• NCBI GI #: 4503117
• NCBI GeneID: 1476
• NCBI Accession #: NP_000091.1
• NCBI GenBank Nucleotide #: NM_000100.3
• Molecular Weight: 11,140 Da
• NCBI Official Full Name: cystatin-B
• NCBI Official Synonym Full Names: cystatin B (stefin B)
• NCBI Official Symbol: CSTB
• NCBI Official Synonym Symbols: PME; ULD; CST6; EPM1; STFB; EPM1A
• NCBI Protein Information: cystatin-B; CPI-B; liver thiol proteinase inhibitor
• UniProt Protein Name: Cystatin-B
• Protein Family: Cystatin
• UniProt Gene Name: CSTB
• UniProt Synonym Gene Names: CST6; STFB
• UniProt Entry Name: CYTB_HUMAN

NCBI Description

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). [provided by RefSeq, Jul 2008]

Uniprot Description

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: extracellular space; cytoplasm; nucleolus

Molecular Function: protease binding; endopeptidase inhibitor activity; cysteine protease inhibitor activity

Biological Process: regulation of apoptosis; negative regulation of proteolysis; adult locomotory behavior; negative regulation of peptidase activity

Disease: Myoclonic Epilepsy Of Unverricht And Lundborg