Product Name
Cystatin B (CSTB), Monoclonal Antibody
Full Product Name
Mouse Anti-Human Cystatin B
Product Synonym Names
CSTB; PME; ULD; CST6; EPM1; STFB; EPM1A
Product Gene Name
anti-CSTB antibody
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Chromosome Location
Chromosome: 21; NC_000021.9 (43773665..43776375, complement). Location: 21q22.3
3D Structure
ModBase 3D Structure for P04080
Antibody Generation
This antibody was produced from a hybridoma (mouse myeloma fused with spleen cells from a mouse) immunized with human recombinant Cystatin-B.
Reconstitution
Reconstitute the antibody with 200 ul sterile PBS and the final concentration is 500 ug/ml.
Preparation and Storage
Lyophilized samples are stable for 2 years from date of receipt when stored at -70 degree C. Reconstituted antibody can be aliquoted and stored frozen at < -20 degree C for at least for six months without detectable loss of activity.
ISO Certification
Manufactured in an ISO 9001:2008 Certified Laboratory.
Other Notes
Small volumes of anti-CSTB antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-CSTB antibody
Cystatin B, also known as Stefin B or liver thiol proteinase inhibitor, is a member of family 1 of the cystatin superfamily. It is a 98 amino acid, intracellular inhibitor regulating the activities of cysteine proteases of the papain family such as Cathepsins B, H and L.
Applications Tested/Suitable for anti-CSTB antibody
Western Blot (WB), Neutr
NCBI/Uniprot data below describe general gene information for CSTB. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000091.1
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NCBI GenBank Nucleotide #
NM_000100.3
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UniProt Primary Accession #
P04080
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UniProt Related Accession #
P04080[Other Products]
Molecular Weight
11,140 Da[Similar Products]
NCBI Official Full Name
cystatin-B
NCBI Official Synonym Full Names
cystatin B (stefin B)
NCBI Official Symbol
CSTB??[Similar Products]
NCBI Official Synonym Symbols
PME; ULD; CST6; EPM1; STFB; EPM1A
??[Similar Products]
NCBI Protein Information
cystatin-B; CPI-B; liver thiol proteinase inhibitor
UniProt Protein Name
Cystatin-B
UniProt Synonym Protein Names
CPI-B; Liver thiol proteinase inhibitor; Stefin-B
UniProt Gene Name
CSTB??[Similar Products]
UniProt Synonym Gene Names
CST6; STFB??[Similar Products]
UniProt Entry Name
CYTB_HUMAN
NCBI Summary for CSTB
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 Comments for CSTB
Function: This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B.
Subunit structure: Able to form dimers stabilized by noncovalent forces.
Subcellular location: Cytoplasm. Nucleus Ref.6.
Involvement in disease: Epilepsy, progressive myoclonic 1 (EPM1) [MIM:254800]: 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.10
Sequence similarities: Belongs to the cystatin family.
Research Articles on CSTB
1. Skull thickening and an increased prevalence of abnormal findings in skeletal radiographs of patients with EPM1 suggest that this condition is connected to defective cystatin B function.
Precautions
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Disclaimer
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