Mouse anti-Human CSTB Monoclonal Antibody | anti-CSTB antibody

CSTB (Cystatin-B, CPI-B, Stefin B, CST6, STFB, Liver Thiol Proteinase Inhibitor, Stefin-B) (PE)

Cat. Num. AAA6157297

• Gene Names: CSTB; PME; ULD; CST6; EPM1; STFB; CPI-B; EPM1A
• Reactivity: Human
• Applications: ELISA, Immunofluorescence, Immunohistochemistry, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: CSTB; Monoclonal Antibody; CSTB (Cystatin-B; CPI-B; Stefin B; CST6; STFB; Liver Thiol Proteinase Inhibitor; Stefin-B) (PE); anti-CSTB antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG1,k
• Clone Number: M2-F1
• Specificity: Recognizes human CSTB.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with R-Phycoerythrin (PE).
• Sequence Length: 609

• Applicable Applications for anti-CSTB antibody: ELISA (EIA), Immunofluorescence (IF), Immunohistochemistry (IHC), Western Blot (WB)
• Application Notes: IF: 10ug/ml; Applications are based on unconjugated antibody.
• Immunogen: Full length recombinant corresponding to aa1-98 from human CSTB (AAH03370.1) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: MMCGAPSATQPATAETQHIADQVRSQLEEKENKKFPVFKAVSFKSQVVAGTNYFIKVHVGDEDFVHLRVFQSLPHENKPLTLSNYQTNKAKHDELTYF
• Conjugate: PE
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: PE conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (36.52kD).)

Western Blot (WB)

(CSTB monoclonal antibody. Western Blot analysis of CSTB expression in A-431.)

Western Blot (WB)

(Western Blot analysis of CSTB expression in transfected 293T cell line by CSTB monoclonal antibody. Lane 1: CSTB transfected lysate (11kD). Lane 2: Non-transfected lysate.)

Immunohistochemistry (IHC)

(Immunoperoxidase of monoclonal antibody to CSTB on formalin-fixed paraffin-embedded human colon tissue. [antibody concentration 5ug/ml].)

Immunofluorescence (IF)

(Immunofluorescence of monoclonal antibody to CSTB on A-431 cell. [antibody concentration 10ug/ml].)

Product Categories/Family for anti-CSTB antibody

Antibodies; Protease Inhibitors

NCBI and Uniprot Product Information

• NCBI GI #: 13097209
• NCBI GeneID: 1476
• UniProt Accession #: P04080
• NCBI Official Full Name: Homo sapiens cystatin B (stefin B), mRNA
• 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: 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