Mouse anti-Human Cystatin B/CSTB Monoclonal Antibody | anti-CSTB antibody

Mouse anti Human Cystatin B/CSTB Monoclonal Antibody

Cat. Num. AAA2543383

• Gene Names: CSTB; PME; ULD; CST6; EPM1; STFB; CPI-B; EPM1A
• Reactivity: Human
• Applications: ELISA
• Synonyms: Cystatin B/CSTB; Monoclonal Antibody; Mouse anti Human Cystatin B/CSTB Monoclonal Antibody; anti-CSTB antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2a
• Sequence Length: 98

• Applicable Applications for anti-CSTB antibody: ELISA (EIA)
• Application Notes: ELISA: 0.5-1 mug/mL. This antibody can be used at 0.5-1 mug/mL with the appropriate secondary reagents to detect Human CSTB. The detection limit for Human CSTB is approximately 0.0195 ng/well.
• Immunogen: Recombinant Human Cystatin B/CSTB protein
• Buffer: This antibody was produced from a hybridoma resulting from the fusion of a mouse myeloma with B cells obtained from a mouse immunized with purified, recombinant Human Cystatin B/CSTB (rh Cystatin B/CSTB; P04080; Met 2-Phe 98). The IgG fraction of the cell culture supernatant was purified by Protein A affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2 degree C- 8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -70 degree C. Preservative-Free. Sodium azide is recommended to avoid contamination (final concentration 0.05%-0.1%). It is toxic to cells and should be disposed of properly. Avoid repeated freeze-thaw cycles.

NCBI and Uniprot Product Information

• NCBI GI #: 4503117
• NCBI GeneID: 1476
• NCBI GenBank Nucleotide #: NM_000100.3
• Molecular Weight: 11,140 Da
• NCBI Official Full Name: cystatin-B
• NCBI Official Synonym Full Names: cystatin B
• NCBI Official Symbol: CSTB
• NCBI Official Synonym Symbols: PME; ULD; CST6; EPM1; STFB; CPI-B; EPM1A
• NCBI Protein Information: cystatin-B
• 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). 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]

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: cytoplasm; extracellular space; nucleolus

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

Biological Process: negative regulation of peptidase activity; negative regulation of proteolysis

Disease: Myoclonic Epilepsy Of Unverricht And Lundborg