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Products:Signal Transduction >> Metabolism >> Amino Acids
Overview
Product name
Anti-CBS antibody
See all CBS products (4) ...
Description
Mouse monoclonal to CBS
Tested applications
WB, IHC-P, ICC/IFmore details
Cross reactivity
Reacts with
Human
Immunogen
Recombinant fragment: MPSETPQAEV GPTGCPHRSG PHSAKGSLEK GSPEDKEAKE PLWIRPDAPS RCTWQLGRPA SESPHHHTAP AKSPKILPDI LKKIGDTPMV RINKIGKKFG , corresponding to amino acids 1-101 of Human CBS
MPSETPQAEV GPTGCPHRSG PHSAKGSLEK GSPEDKEAKE PLWIRPDAPS RCTWQLGRPA SESPHHHTAP AKSPKILPDI LKKIGDTPMV RINKIGKKFG
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage buffer
Preservative: None
PBS, pH 7.2
Concentration
Concentration information loading...
Purity
Protein G purified
Clonality
Monoclonal
Isotype
IgG2a
Light chain type
kappa
Research areas
Applications
Show applications key
Our Abpromise guarantee covers the use of ab54883 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
WB: Use a concentration of 1 - 5 µg/ml.Predicted molecular weight: 60 kDa.
IHC-P: Use a concentration of 3 µg/ml
ICC/IF: Use a concentration of 10 µg/ml
Target
Function
Only known pyridoxal phosphate-dependent enzyme that contains heme. Important regulator of hydrogen sulfide, especially in the brain, utilizing cysteine instead of serine to catalyze the formation of hydrogen sulfide. Hydrogen sulfide is a gastratransmitter with signaling and cytoprotective effects such as acting as a neuromodulator in the brain to protect neurons against hypoxic injury.
Tissue specificity
In the adult strongly expressed in liver and pancreas, some expression in heart and brain, weak expression in lung and kidney. In the fetus, expressed in brain, liver and kidney.
Pathway
Amino-acid biosynthesis; L-cysteine biosynthesis; L-cysteine from L-homocysteine and L-serine: step 1/2.
Involvement in disease
Defects in CBS are the cause of cystathionine beta-synthase deficiency (CBSD) [MIM:236200]. CBSD is an enzymatic deficiency resulting in altered sulfur metabolism and homocystinuria. The clinical features of untreated homocystinuria due to CBS deficiency include myopia, ectopia lentis, mental retardation, skeletal anomalies resembling Marfan syndrome, and thromboembolic events. Light skin and hair can also be present. Biochemical features include increased urinary homocystine and methionine.
Sequence similarities
Belongs to the cysteine synthase/cystathionine beta-synthase family.
Contains 1 CBS domain.
Cellular localization
Cytoplasm. Nucleus.
Target information above from: UniProt accessionP35520
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
Alternative names
- Beta thionase antibody
- Beta-thionase antibody
- Cbs antibody
see all
Database links
- Entrez Gene: 875 Human
- Omim: 236200 Human
- SwissProt: P35520 Human
- Unigene: 533013 Human
Anti-CBS antibody images:
IHC-P - CBS antibody (ab54883)
CBS antibody (ab54883) used in immunohistochemistry at 3ug/ml on formalin fixed and paraffin embedded human hepatocellular carcinoma.
Western blot - CBS antibody (ab54883)
CBS antibody (ab54883) at 1ug/lane + HeLa cell lysate at 25ug/lane.
Immunocytochemistry/ Immunofluorescence-CBS antibody(ab54883)
ICC/IF image of ab54883 stained HeLa cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab54883, 10µg/ml) overnight at +4°C. The secondary antibody (green) was ab96879, DyLight® 488 goat anti-mouse IgG (H+L) used at a 1/250 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.
References for Anti-CBS antibody (ab54883)
ab54883 has not yet been referenced specifically in any publications.
Publishing research using ab54883? Please let us know so that we can cite the reference in this datasheet
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"