Cystathionine beta Synthase Assay Kit (ab241043)
Key features and details
- Detection method: Fluorescent
- Platform: Microplate reader
- Sample type: Adherent cells, Cell culture supernatant, Suspension cells, Tissue
Overview
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Product name
Cystathionine beta Synthase Assay Kit
See all CBS kits -
Detection method
Fluorescent -
Sample type
Cell culture supernatant, Tissue, Adherent cells, Suspension cells -
Product overview
Cystathionine beta Synthase Assay Kit (ab241043) utilizes cysteine and homocysteine as substrates to produce H2S. Hydrogen sulfide reacts with the azido-functional group of the fluorescent probe yielding a fluorescent amino group (Ex/Em = 368/460 nm). The assay is highly sensitive, has a simple easy-to-follow protocol, and can detect as low as 1.45 mU of CβS activity.
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Platform
Microplate reader
Properties
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Storage instructions
Store at -20°C. Please refer to protocols. -
Components Identifier 100 tests AMC Standard (in DMSO, 1 mM) Yellow 1 x 100µl CβS Assay Buffer WM 1 x 25ml CβS Positive Control Green 1 x 50µl CβS Probe (in DMSO) Purple 1 x 500µl CβS Substrate Amber NM 1 x 4ml Cofactor 1 Amber 1 x 500µl Cofactor 2 Orange 1 x 500µl Reducing Agent (20X) Cap colour Amber/Yellow 1 vial -
Research areas
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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. - Information by UniProt
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Alternative names
- AI047524
- AI303044
- Beta thionase
see all