3-mercaptopyruvate sulfurtransferase (MPST)
The protein contains 297 amino acids for an estimated molecular weight of 33178 Da.
Transfer of a sulfur ion to cyanide or to other thiol compounds. Also has weak rhodanese activity. Detoxifies cyanide and is required for thiosulfate biosynthesis. Acts as an antioxidant. In combination with cysteine aminotransferase (CAT), contributes to the catabolism of cysteine and is an important producer of hydrogen sulfide in the brain, retina and vascular endothelial cells. Hydrogen sulfide H(2)S is an important synaptic modulator, signaling molecule, smooth muscle contractor and neuroprotectant. Its production by the 3MST/CAT pathway is regulated by calcium ions. (updated: March 28, 2018)
Protein identification was indicated in the following studies:
- Goodman and co-workers. (2013) The proteomics and interactomics of human erythrocytes. Exp Biol Med (Maywood) 238(5), 509-518.
- Lange and co-workers. (2014) Annotating N termini for the human proteome project: N termini and Nα-acetylation status differentiate stable cleaved protein species from degradation remnants in the human erythrocyte proteome. J Proteome Res. 13(4), 2028-2044.
- Hegedűs and co-workers. (2015) Inconsistencies in the red blood cell membrane proteome analysis: generation of a database for research and diagnostic applications. Database (Oxford) 1-8.
- Bryk and co-workers. (2017) Quantitative Analysis of Human Red Blood Cell Proteome. J Proteome Res. 16(8), 2752-2761.
- D'Alessandro and co-workers. (2017) Red blood cell proteomics update: is there more to discover? Blood Transfus. 15(2), 182-187.
Methods
The following articles were analysed to gather the proteome content of erythrocytes.
The gene or protein list provided in the studies were processed using the ID mapping API of Uniprot in September 2018. The number of proteins identified and mapped without ambiguity in these studies is indicated below.
Only Swiss-Prot entries (reviewed) were considered for protein evidence assignation.
| Publication | Identification 1 | Uniprot mapping 2 | Not mapped / Obsolete | TrEMBL | Swiss-Prot |
|---|---|---|---|---|---|
| Goodman (2013) | 2289 (gene list) | 2278 | 53 | 20599 | 2269 |
| Lange (2014) | 1234 | 1234 | 7 | 28 | 1224 |
| Hegedus (2015) | 2638 | 2622 | 0 | 235 | 2387 |
| Wilson (2016) | 1658 | 1528 | 170 | 291 | 1068 |
| d'Alessandro (2017) | 1826 | 1817 | 2 | 0 | 1815 |
| Bryk (2017) | 2090 | 2060 | 10 | 108 | 1942 |
| Chu (2018) | 1853 | 1804 | 55 | 362 | 1387 |
1 as available in the article and/or in supplementary material
2 uniprot mapping returns all protein isoforms as one entry
The compilation of older studies can be retrieved from the Red Blood Cell Collection database.
The data and differentiation stages presented below come from the proteomic study and analysis performed by our partners of the GReX consortium, more details are available in their published work.
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Biological Process
Cyanate catabolic process
Hydrogen sulfide biosynthetic process
Kidney development
Liver development
Response to toxic substance
Spinal cord development
Sulfur amino acid catabolic process
Transsulfuration
The reference OMIM entry for this protein is 249650
Mercaptolactate-cysteine disulfiduria; mcdu
Disulfiduria, mixed
CLINICAL FEATURES
Ampola et al. (1969) described a placid, hypokinetic, 45-year-old male, the product of brother-sister incest, who had a low IQ, grand mal seizures, flattened nasal bridge, and excessively arched palate. He was found in the course of screening mentally retarded patients with the nitroprusside test. He excreted large amounts of a sulfur-containing amino acid, shown by Crawhall et al. (1969) to be beta-mercaptolactate-cysteine disulfide. The structure of the disulfide is indicated by its name: in one-half of the molecule the-NH2 of cysteine is replaced by-OH. Administration of cysteine, but not of methionine, increased excretion of the mixed disulfide (Crawhall et al., 1971). During a screening for cystinuria in the regular schools, Niederwieser et al. (1973) found the same material in the urine of 2 mentally normal sisters, aged 11 and 13 years. In Sweden, Hannestad et al. (1981) detected MCDU in a mentally retarded woman. Her red cells were devoid of activity of the enzyme mercaptopyruvate sulfurtransferase (MPST; 602496). Both parents and 5 other relatives in a pattern consistent with their being heterozygotes showed intermediate levels of red cell MST. The presumed heterozygotes also excreted excessive amounts of mercaptolactate and mercaptoacetate in the urine. ... More on the omim web site
April 12, 2018: Protein entry updated
Automatic update: Entry updated from uniprot information.
Feb. 2, 2018: Protein entry updated
Automatic update: Uniprot description updated
Dec. 19, 2017: Protein entry updated
Automatic update: Uniprot description updated
Nov. 23, 2017: Protein entry updated
Automatic update: Uniprot description updated
June 20, 2017: Protein entry updated
Automatic update: comparative model was added.
March 15, 2016: Protein entry updated
Automatic update: OMIM entry 249650 was added.
Jan. 27, 2016: Protein entry updated
Automatic update: model status changed
Jan. 24, 2016: Protein entry updated
Automatic update: model status changed