Human Cystatin B ELISA Kit | CSTB elisa kit

Human Cystatin B PicoKine ELISA Kit

Cat. Num. AAA177260

• Gene Names: CSTB; PME; ULD; CST6; EPM1; STFB; EPM1A
• Reactivity: Human
• Synonyms: Cystatin B; Human Cystatin B PicoKine ELISA Kit; Cystatin-B; cystatin B (stefin B); CHROW21; CPI B; CPI-B; CST6; CSTB; CYTB; CYTB_HUMAN; EPM1; EPM1A; Liver thiol proteinase inhibitor; PME; Stefin-B; STFB; ULD; CSTB elisa kit

• Reactivity: Human
• Specificity: Natural and recombinant human Cystatin B
• Sequence Length: 98

• Samples: Cell culture supernates, serum, plasma (heparin, EDTA), saliva, urine and human milk
• Assay Type: Sandwich
• Detection Range: 62.5pg/ml-4000pg/ml
• Sensitivity: < 10pg/ml
• Intra-assay Precision: Intra-Assay Precision (Precision within an assay) Three samples of known concentration were tested on one plate to assess intra-assay precision.
• Inter-assay Precision: Inter-Assay Precision (Precision between assays) Three samples of known concentration were tested in separate assays to assess inter-assay precision.
• Preparation and Storage: Store at 4 degree C for 6 months, at -20 degree C for 12 months. Avoid multiple freeze-thaw cycles (Shipped with wet ice.)

Typical Testing Data/Standard Curve (for reference only)

(Human Cystatin B PicoKine ELISA Kit standard curve )

Related Product Information for CSTB elisa kit

Principle of the assay: human Cystatin B ELISA Kit was based on standard sandwich enzyme-linked immune-sorbent assay technology. A monoclonal antibody from mouse specific for Cystatin B has been precoated onto 96-well plates. Standards(E.coli, M2-F98) and test samples are added to the wells, a biotinylated detection polyclonal antibody from goat specific for Cystatin B is added subsequently and then followed by washing with PBS or TBS buffer. Avidin-Biotin-Peroxidase Complex was added and unbound conjugates were washed away with PBS or TBS buffer. HRP substrate TMB was used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the human Cystatin B amount of sample captured in plate.
Background: Cystatin B(CSTB), also called STFB, is a small protein that is a member of the superfamily of cysteine protease inhibitors. It has been isolated from human spleen and liver and its amino acid sequence has been fully determined. The cystatin B gene is located on 21q22.3. It is widely distributed and is localized mostly intracellularly, but has been found extracellularly. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. Its role is thought to be as a protector against the proteinases leaking from lysosomes. A cystatin B multiprotein complex might have a specific cerebellar function, and that the loss of this function might contribute to the etiopathogenesis of EPM1. Upon differentiation to myotubes, CSTB becomes excluded from the nucleus and lysosomes, suggesting that the subcellular distribution of CSTB is dependent on the differentiation status of the cell.

NCBI and Uniprot Product Information

• NCBI GI #: 1706278
• NCBI GeneID: 1476
• UniProt Accession #: P04080
• 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: negative regulation of proteolysis; regulation of apoptosis; adult locomotory behavior; negative regulation of peptidase activity

Disease: Myoclonic Epilepsy Of Unverricht And Lundborg