TST (Thiosulfate Sulfurtransferase)
Gene encoding rhodanese, a mitochondrial enzyme involved in cyanide detoxification and sulfur metabolism.
Gene Information Card
| Symbol | TST |
|---|---|
| Full Name | Thiosulfate Sulfurtransferase |
| Gene Type | protein-coding |
| Chromosomal Location | 22q12.3 |
| NCBI Gene ID | 7263 ncbi.nlm.nih.gov/gene/7263 |
| Ensembl ID | ENSG00000128311 |
| UniProt ID | Q16762 |
| OMIM ID | 180450 |
| HGNC ID | 12425 |
| Aliases | RDS, RDS1, thiosulfate sulfurtransferase, rhodanese |
Description
The TST gene encodes thiosulfate sulfurtransferase, also known as rhodanese, a mitochondrial matrix enzyme that catalyzes the transfer of a sulfur atom from thiosulfate to cyanide, forming thiocyanate and sulfite. This reaction is a key pathway for cyanide detoxification. The enzyme also participates in sulfur metabolism and may have roles in mitochondrial function and oxidative stress response.
Disease Associations
| Disease category | Pathophysiological mechanism | Genomic evidence |
|---|---|---|
| Cyanide poisoning | Deficiency in TST reduces cyanide detoxification capacity, leading to toxicity. | PMID: 23867502 |
| Ethylmalonic encephalopathy | TST dysfunction may contribute to sulfur metabolism defects and mitochondrial dysfunction. | PMID: 25255310 |
| Leber hereditary optic neuropathy (LHON) | Altered TST expression linked to mitochondrial dysfunction in LHON. | PMID: 20041218 |
Expression Profile
Tissue Expression
| Tissue | nTPM | level |
|---|---|---|
| Liver | 12.3 | Medium |
| Kidney | 8.7 | Medium |
| Heart | 6.2 | Low |
| Brain | 4.5 | Low |
| Lung | 3.1 | Low |
Cell Line Expression
| Cell Line | nTPM | Notes |
|---|---|---|
| HepG2 | 10.1 | Hepatocellular carcinoma cell line |
| HEK293 | 7.8 | Embryonic kidney cells |
| SH-SY5Y | 5.4 | Neuroblastoma cell line |
| A549 | 4.2 | Lung carcinoma cell line |
Data source：Human Protein Atlas（proteinatlas.org）
Mutations & Variants
Hotspot Mutations
| Variant | Type | Frequency | Functional Description |
|---|---|---|---|
| c.1A>G (p.Met1?) | missense | <0.01% | Likely loss of function; start codon loss |
| c.374C>T (p.Thr125Met) | missense | <0.01% | Uncertain significance; may affect enzyme activity |
| c.589G>A (p.Gly197Arg) | missense | <0.01% | Uncertain significance; reported in ClinVar |
Mutation functional classification
Loss of Function (LOF)
Loss-of-function mutations in TST reduce rhodanese activity, impairing cyanide detoxification and sulfur metabolism.
Gain of Function (GOF)
No gain-of-function mutations reported.
Dominant Negative (DN)
No dominant-negative mutations reported.
View complete mutation data：
Gene Ontology (GO)
Pathways
• Cyanide detoxification (Reactome: R-HSA-9014325)
• Sulfur metabolism (KEGG: hsa00920)
Protein Summary
Thiosulfate sulfurtransferase (rhodanese) is a 33 kDa mitochondrial enzyme encoded by the TST gene. It catalyzes the transfer of a sulfur atom from thiosulfate to cyanide, producing thiocyanate and sulfite, a critical step in cyanide detoxification. The enzyme contains a conserved rhodanese domain and is expressed in various tissues, with highest levels in liver and kidney. It also participates in sulfur metabolism and may protect against oxidative stress.
Related Services
Related Products
| Product name | Cat.No. | Species | Gene ID | |
|---|---|---|---|---|
| TSTD1 Knockout HEK293 Cell Line | EDJ-KQ11956 | Human | 100131187 | Details Get a Quote |
| TSTD2 Knockout HEK293 Cell Line | EDJ-KQ15964 | Human | 158427 | Details Get a Quote |
| TST Knockout HEK293 Cell Line | EDJ-KQ17233 | Human | 7263 | Details Get a Quote |
| TST Knockout HCT 116 Cell Line | EDJ-KQ17921 | Human | 7263 | Details Get a Quote |
| TSTD2 Knockout A-549 Cell Line | EDJ-KQ46994 | Human | 158427 | Details Get a Quote |
| TSTD2 Knockout HCT 116 Cell Line | EDJ-KQ46995 | Human | 158427 | Details Get a Quote |
| TSTD2 Knockout HeLa Cell Line | EDJ-KQ46996 | Human | 158427 | Details Get a Quote |
| TST Knockout A-549 Cell Line | EDJ-KQ49210 | Human | 7263 | Details Get a Quote |
| TST Knockout HeLa Cell Line | EDJ-KQ49211 | Human | 7263 | Details Get a Quote |
| TSTD1 Knockout HCT 116 Cell Line | EDJ-KQ40488 | Human | 100131187 | Details Get a Quote |
| TSTD1 Knockout HeLa Cell Line | EDJ-KQ40489 | Human | 100131187 | Details Get a Quote |
| TSTD1 Knockout A-549 Cell Line | EDJ-KQ69290 | Human | 100131187 | Details Get a Quote |
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