CBS
cystathionine beta-synthase | HIP4

The protein encoded by this gene acts as a homotetramer to catalyze the conversion of homocysteine to cystathionine, the first step in the transsulfuration pathway. The encoded protein is allosterically activated by adenosyl-methionine and uses pyridoxal phosphate as a cofactor. Defects in this gene can cause cystathionine beta-synthase deficiency (CBSD), which can lead to homocystinuria. This gene is a major contributor to cellular hydrogen sulfide production. Multiple alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2016]

Member of: DE-1 DE-1.55 Developmental clusters: GC1
Biological processes 45 terms
DNA protection (GO:0042262)L-cysteine biosynthetic process (GO:0019344)L-cysteine biosynthetic process (GO:0019344)L-cysteine biosynthetic process (GO:0019344)L-cysteine catabolic process (GO:0019448)L-homocysteine catabolic process (GO:0043418)L-homocysteine catabolic process (GO:0043418)L-serine catabolic process (GO:0006565)L-serine metabolic process (GO:0006563)S-adenosyl-L-methionine binding (GO:1904047)carbon monoxide binding (GO:0070025)cellular response to hypoxia (GO:0071456)cellular response to insulin stimulus (GO:0032869)cystathionine beta-synthase activity (GO:0004122)cystathionine beta-synthase activity (GO:0004122)cystathionine beta-synthase activity (GO:0004122)cytoplasm (GO:0005737)cytoplasm (GO:0005737)cytoplasm (GO:0005737)cytosol (GO:0005829)cytosol (GO:0005829)enzyme binding (GO:0019899)heme binding (GO:0020037)heme binding (GO:0020037)heme binding (GO:0020037)homocysteine metabolic process (GO:0050667)homocysteine metabolic process (GO:0050667)hydrogen sulfide biosynthetic process (GO:0070814)hydrogen sulfide biosynthetic process (GO:0070814)hydrogen sulfide biosynthetic process (GO:0070814)identical protein binding (GO:0042802)modified amino acid binding (GO:0072341)negative regulation of apoptotic process (GO:0043066)nitric oxide binding (GO:0070026)nitrite reductase (NO-forming) activity (GO:0050421)nucleus (GO:0005634)nucleus (GO:0005634)oxygen binding (GO:0019825)protein binding (GO:0005515)protein homodimerization activity (GO:0042803)pyridoxal phosphate binding (GO:0030170)response to nutrient levels (GO:0031667)sulfur compound biosynthetic process (GO:0044272)transsulfuration (GO:0019346)ubiquitin protein ligase binding (GO:0031625)
Expression (TPM)
CBS — as a Regulated Gene

TFs regulating CBS 0 TFs

Transcription factors with Perturb-seq knockdown data for CBS. The Binding column indicates whether any binding evidence exists for this TF–gene pair (ChIP-seq or motif footprint peaks). The Mean coef is the average Perturb-seq regression coefficient across active gRNAs (positive = CBS upregulated upon KD; negative = downregulated). The Outlier column indicates whether this gene is in the top or bottom 5% of all TF knockdown effects.

Data: Effect:
TF Mean coef Binding Outlier TF→Gene link

Elements linked to CBS

Open chromatin peaks (ATAC-seq) in the genomic neighbourhood of CBS, linked by TSS proximity or chromatin conformation (Multiome / HiCAR). Each element overlaps at least one TF ChIP-seq binding site — the TFs column shows how many distinct TFs bind that element.

Accessibility Element Dist. to TSS Link type TFs
chr21:42,793,699–42,794,977 281.9 kb Distal (>10kb) Multiome 52
chr21:42,806,044–42,807,071 269.3 kb Distal (>10kb) Multiome 113
chr21:42,878,621–42,880,078 196.5 kb Distal (>10kb) Multiome 989
chr21:42,892,360–42,893,974 182.8 kb Distal (>10kb) Multiome 992
chr21:42,923,670–42,924,528 151.9 kb Distal (>10kb) Multiome 542
chr21:42,925,494–42,925,953 150.2 kb Distal (>10kb) Multiome 42
chr21:42,936,514–42,937,904 138.4 kb Distal (>10kb) Multiome 182
chr21:42,955,489–42,956,014 120.2 kb Distal (>10kb) Multiome 345
chr21:42,956,807–42,957,632 118.7 kb Distal (>10kb) Multiome 172
chr21:42,972,793–42,975,770 101.5 kb Distal (>10kb) Multiome 945

Genome Browser

Genomic view of the CBS locus showing ATAC-seq accessibility and RNA-seq expression across the ESC → DE time course, together with TF binding peaks and element-to-TSS loop connections.

chr21:42,783,699 – 42,985,770
Proximal 1 kb Distal 10 kb Multiome HiCAR ATAC-seq RNA-seq