| Identification |
|---|
| HMDB Protein ID
| CDBP00724 |
| Secondary Accession Numbers
| Not Available |
| Name
| Cystathionine beta-synthase |
| Description
| Not Available |
| Synonyms
|
- Beta-thionase
- Serine sulfhydrase
|
| Gene Name
| CBS |
| Protein Type
| Enzyme |
| Biological Properties |
|---|
| General Function
| Involved in cysteine biosynthetic process from serine |
| Specific 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 (By similarity).
|
| GO Classification
|
| Biological Process |
| cerebellum morphogenesis |
| cysteine biosynthetic process from serine |
| cysteine biosynthetic process via cystathionine |
| endochondral ossification |
| homocysteine catabolic process |
| hydrogen sulfide biosynthetic process |
| L-serine catabolic process |
| L-cysteine catabolic process |
| regulation of cGMP metabolic process |
| regulation of JUN kinase activity |
| response to folic acid |
| superoxide metabolic process |
| transsulfuration |
| cellular nitrogen compound metabolic process |
| regulation of blood vessel size |
| blood vessel remodeling |
| maternal process involved in female pregnancy |
| cartilage development involved in endochondral bone morphogenesis |
| Cellular Component |
| cytosol |
| nucleolus |
| Component |
| cell part |
| intracellular part |
| cytoplasm |
| Function |
| hydro-lyase activity |
| cofactor binding |
| pyridoxal phosphate binding |
| cystathionine beta-synthase activity |
| binding |
| catalytic activity |
| lyase activity |
| carbon-oxygen lyase activity |
| Molecular Function |
| metal ion binding |
| pyridoxal phosphate binding |
| heme binding |
| protein homodimerization activity |
| cystathionine beta-synthase activity |
| modified amino acid binding |
| Process |
| cysteine biosynthetic process via cystathionine |
| l-serine metabolic process |
| cysteine biosynthetic process from serine |
| cellular amino acid and derivative metabolic process |
| cellular amino acid metabolic process |
| metabolic process |
| cellular metabolic process |
| sulfur amino acid metabolic process |
| sulfur amino acid biosynthetic process |
| serine family amino acid metabolic process |
| cysteine biosynthetic process |
|
| Cellular Location
|
- Nucleus
- Cytoplasm
|
| Pathways
|
| Name | SMPDB/Pathwhiz | KEGG | | L-cysteine biosynthesis | Not Available | Not Available | | Glycine, serine and threonine metabolism | Not Available |  | | Cysteine and methionine metabolism | Not Available |  | | Selenoamino Acid Metabolism |    |  | | Homocysteine Degradation |    | Not Available |
|
| Gene Properties |
|---|
| Chromosome Location
| 21 |
| Locus
| 21q22.3 |
| SNPs
| CBS |
| Gene Sequence
|
>1656 bp
ATGCCTTCTGAGACCCCCCAGGCAGAAGTGGGGCCCACAGGCTGCCCCCACCGCTCAGGG
CCACACTCGGCGAAGGGGAGCCTGGAGAAGGGGTCCCCAGAGGATAAGGAAGCCAAGGAG
CCCCTGTGGATCCGGCCCGATGCTCCGAGCAGGTGCACCTGGCAGCTGGGCCGGCCTGCC
TCCGAGTCCCCACATCACCACACTGCCCCGGCAAAATCTCCAAAAATCTTGCCAGATATT
CTGAAGAAAATCGGGGACACCCCTATGGTCAGAATCAACAAGATTGGGAAGAAGTTCGGC
CTGAAGTGTGAGCTCTTGGCCAAGTGTGAGTTCTTCAACGCGGGCGGGAGCGTGAAGGAC
CGCATCAGCCTGCGGATGATTGAGGATGCTGAGCGCGACGGGACGCTGAAGCCCGGGGAC
ACGATTATCGAGCCGACATCCGGGAACACCGGGATCGGGCTGGCCCTGGCTGCGGCAGTG
AGGGGCTATCGCTGCATCATCGTGATGCCAGAGAAGATGAGCTCCGAGAAGGTGGACGTG
CTGCGGGCACTGGGGGCTGAGATTGTGAGGACGCCCACCAATGCCAGGTTCGACTCCCCG
GAGTCACACGTGGGGGTGGCCTGGCGGCTGAAGAACGAAATCCCCAATTCTCACATCCTA
GACCAGTACCGCAACGCCAGCAACCCCCTGGCTCACTACGACACCACCGCTGATGAGATC
CTGCAGCAGTGTGATGGGAAGCTGGACATGCTGGTGGCTTCAGTGGGCACGGGCGGCACC
ATCACGGGCATTGCCAGGAAGCTGAAGGAGAAGTGTCCTGGATGCAGGATCATTGGGGTG
GATCCCGAAGGGTCCATCCTCGCAGAGCCGGAGGAGCTGAACCAGACGGAGCAGACAACC
TACGAGGTGGAAGGGATCGGCTACGACTTCATCCCCACGGTGCTGGACAGGACGGTGGTG
GACAAGTGGTTCAAGAGCAACGATGAGGAGGCGTTCACCTTTGCCCGCATGCTGATCGCG
CAAGAGGGGCTGCTGTGCGGTGGCAGTGCTGGCAGCACGGTGGCGGTGGCCGTGAAGGCT
GCGCAGGAGCTGCAGGAGGGCCAGCGCTGCGTGGTCATTCTGCCCGACTCAGTGCGGAAC
TACATGACCAAGTTCCTGAGCGACAGGTGGATGCTGCAGAAGGGCTTTCTGAAGGAGGAG
GACCTCACGGAGAAGAAGCCCTGGTGGTGGCACCTCCGTGTTCAGGAGCTGGGCCTGTCA
GCCCCGCTGACCGTGCTCCCGACCATCACCTGTGGGCACACCATCGAGATCCTCCGGGAG
AAGGGCTTCGACCAGGCGCCCGTGGTGGATGAGGCGGGGGTAATCCTGGGAATGGTGACG
CTTGGGAACATGCTCTCGTCCCTGCTTGCCGGGAAGGTGCAGCCGTCAGACCAAGTTGGC
AAAGTCATCTACAAGCAGTTCAAACAGATCCGCCTCACGGACACGCTGGGCAGGCTCTCG
CACATCCTGGAGATGGACCACTTCGCCCTGGTGGTGCACGAGCAGATCCAGTACCACAGC
ACCGGGAAGTCCAGTCAGCGGCAGATGGTGTTCGGGGTGGTCACCGCCATTGACTTGCTG
AACTTCGTGGCCGCCCAGGAGCGGGACCAGAAGTGA
|
| Protein Properties |
|---|
| Number of Residues
| 551 |
| Molecular Weight
| 60586.05 |
| Theoretical pI
| 6.648 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>Cystathionine beta-synthase
MPSETPQAEVGPTGCPHRSGPHSAKGSLEKGSPEDKEAKEPLWIRPDAPSRCTWQLGRPA
SESPHHHTAPAKSPKILPDILKKIGDTPMVRINKIGKKFGLKCELLAKCEFFNAGGSVKD
RISLRMIEDAERDGTLKPGDTIIEPTSGNTGIGLALAAAVRGYRCIIVMPEKMSSEKVDV
LRALGAEIVRTPTNARFDSPESHVGVAWRLKNEIPNSHILDQYRNASNPLAHYDTTADEI
LQQCDGKLDMLVASVGTGGTITGIARKLKEKCPGCRIIGVDPEGSILAEPEELNQTEQTT
YEVEGIGYDFIPTVLDRTVVDKWFKSNDEEAFTFARMLIAQEGLLCGGSAGSTVAVAVKA
AQELQEGQRCVVILPDSVRNYMTKFLSDRWMLQKGFLKEEDLTEKKPWWWHLRVQELGLS
APLTVLPTITCGHTIEILREKGFDQAPVVDEAGVILGMVTLGNMLSSLLAGKVQPSDQVG
KVIYKQFKQIRLTDTLGRLSHILEMDHFALVVHEQIQYHSTGKSSQRQMVFGVVTAIDLL
NFVAAQERDQK
|
| External Links |
|---|
| GenBank ID Protein
| 388716 |
| UniProtKB/Swiss-Prot ID
| P35520 |
| UniProtKB/Swiss-Prot Entry Name
| CBS_HUMAN |
| PDB IDs
|
|
| GenBank Gene ID
| L19501 |
| GeneCard ID
| CBS |
| GenAtlas ID
| CBS |
| HGNC ID
| HGNC:1550 |
| References |
|---|
| General References
| Not Available |