Recombinant Human CBS protein (ab132564)
- Datasheet
- References
- Protocols
Overview
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Product name
Recombinant Human CBS protein
See all CBS proteins and peptides -
Protein length
Full length protein
Description
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Nature
Recombinant -
Source
Wheat germ -
Amino Acid Sequence
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Accession
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Species
Human -
Sequence
MPSETPQAEVGPTGCPHRSGPHSAKGSLEKGSPEDKEAKEPLWIRPDAPS RCTWQLGRPASESPHHHTAPAKSPKILPDILKKIGDTPMVRINKIGKKFG LKCELLAKCEFFNAGGSVKDRISLRMIEDAERDGTLKPGDTIIEPTSGNT GIGLALAAAVRGYRCIIVMPEKMSSEKVDVLRALGAEIVRTPTNARFDSP ESHVGVAWRLKNEIPNSHILDQYRNASNPLAHYDTTADEILQQCDGKLDM LVASVGTGGTITGIARKLKEKCPGCRIIGVDPEGSILAEPEELNQTEQTT YEVEGIGYDFIPTVLDRTVVDKWFKSNDEEAFTFARMLIAQEGLLCGGSA GSTVAVAVKAAQELQEGQRCVVILPDSVRNYMTKFLSDRWMLQKGFLKEE DLTEKKPWWWHLRVQELGLSAPLTVLPTITCGHTIEILREKGFDQAPVVD EAGVILGMVTLGNMLSSLLAGKVQPSDQVGKVIYKQFKQIRLTDTLGRLS HILEMDHFALVVHEQIQYHSTGKSSQRQMVFGVVTAIDLLNFVAAQERDQ K -
Molecular weight
86 kDa including tags -
Amino acids
1 to 551 -
Tags
GST tag N-Terminus
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Associated products
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Related Products
Specifications
Our Abpromise guarantee covers the use of ab132564 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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Applications
ELISA
SDS-PAGE
Western blot
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Form
Liquid -
Additional notes
Protein concentration is above or equal to 0.05 µg/µl. -
Concentration information loading...
Preparation and Storage
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Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00
Constituents: 0.31% Glutathione, 0.79% Tris HCl
General Info
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Alternative names
- AI047524
- AI303044
- Beta thionase
see all -
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
Images
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SDS-PAGE - Recombinant Human CBS protein (ab132564)12.5% SDS-PAGE analysis of ab132564 stained with Coomassie Blue.
Datasheets and documents
References
ab132564 has not yet been referenced specifically in any publications.
