{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T01:41:45Z","timestamp":1790041305146,"version":"4.0.1"},"reference-count":49,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2020,1,27]],"date-time":"2020-01-27T00:00:00Z","timestamp":1580083200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["16H05107"],"award-info":[{"award-number":["16H05107"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["17K08287"],"award-info":[{"award-number":["17K08287"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Mercaptopyruvate sulfurtransferase (Mpst) and its homolog thiosulfate sulfurtransferase (Tst = rhodanese) detoxify cyanide to thiocyanate. Mpst is attracting attention as one of the four endogenous hydrogen sulfide (H2S)\/reactive sulfur species (RSS)-producing enzymes, along with cystathionine \u03b2-synthase (Cbs), cystathionine \u03b3-lyase (Cth), and cysteinyl-tRNA synthetase 2 (Cars2). MPST deficiency was found in 1960s among rare hereditary mercaptolactate-cysteine disulfiduria patients. Mpst-knockout (KO) mice with enhanced liver Tst expression were recently generated as its model; however, the physiological roles\/significances of Mpst remain largely unknown. Here we generated three independent germ lines of Mpst-KO mice by CRISPR\/Cas9 technology, all of which maintained normal hepatic Tst expression\/activity. Mpst\/Cth-double knockout (DKO) mice were generated via crossbreeding with our previously generated Cth-KO mice. Mpst-KO mice were born at the expected frequency and developed normally like Cth-KO mice, but displayed increased urinary 3-mercaptolactate excretion and enhanced passive systemic anaphylactic responses when compared to wild-type or Cth-KO mice. Mpst\/Cth-DKO mice were also born at the expected frequency and developed normally, but excreted slightly more 3-mercaptolactate in urine compared to Mpst-KO or Cth-KO mice. Our Mpst-KO, Cth-KO, and Mpst\/Cth-DKO mice, unlike semi-lethal Cbs-KO mice and lethal Cars2-KO mice, are useful tools for analyzing the unknown physiological roles of endogenous H2S\/RSS production.<\/jats:p>","DOI":"10.3390\/ijms21030818","type":"journal-article","created":{"date-parts":[[2020,1,27]],"date-time":"2020-01-27T11:41:57Z","timestamp":1580125317000},"page":"818","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Increased Urinary 3-Mercaptolactate Excretion and Enhanced Passive Systemic Anaphylaxis in Mice Lacking Mercaptopyruvate Sulfurtransferase, a Model of Mercaptolactate-Cysteine Disulfiduria"],"prefix":"10.3390","volume":"21","author":[{"given":"Noriyuki","family":"Akahoshi","sequence":"first","affiliation":[{"name":"Department of Health Chemistry, Showa Pharmaceutical University, Machida, Tokyo 194-8543, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatsuro","family":"Minakawa","sequence":"additional","affiliation":[{"name":"Department of Health Chemistry, Showa Pharmaceutical University, Machida, Tokyo 194-8543, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masashi","family":"Miyashita","sequence":"additional","affiliation":[{"name":"Department of Health Chemistry, Showa Pharmaceutical University, Machida, Tokyo 194-8543, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uran","family":"Sugiyama","sequence":"additional","affiliation":[{"name":"Department of Health Chemistry, Showa Pharmaceutical University, Machida, Tokyo 194-8543, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chihiro","family":"Saito","sequence":"additional","affiliation":[{"name":"Department of Health Chemistry, Showa Pharmaceutical University, Machida, Tokyo 194-8543, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rintaro","family":"Takemoto","sequence":"additional","affiliation":[{"name":"Department of Health Chemistry, Showa Pharmaceutical University, Machida, Tokyo 194-8543, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akihiro","family":"Honda","sequence":"additional","affiliation":[{"name":"Department of Health Chemistry, Showa Pharmaceutical University, Machida, Tokyo 194-8543, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Waka","family":"Kamichatani","sequence":"additional","affiliation":[{"name":"Department of Health Chemistry, Showa Pharmaceutical University, Machida, Tokyo 194-8543, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shotaro","family":"Kamata","sequence":"additional","affiliation":[{"name":"Department of Health Chemistry, Showa Pharmaceutical University, Machida, Tokyo 194-8543, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasumi","family":"Anan","sequence":"additional","affiliation":[{"name":"Department of Health Chemistry, Showa Pharmaceutical University, Machida, Tokyo 194-8543, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5367-205X","authenticated-orcid":false,"given":"Isao","family":"Ishii","sequence":"additional","affiliation":[{"name":"Department of Health Chemistry, Showa Pharmaceutical University, Machida, Tokyo 194-8543, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,1,27]]},"reference":[{"key":"ref_1","first-page":"66","article-title":"Mental deficiency and a new aminoaciduria","volume":"117","author":"Ampola","year":"1969","journal-title":"Am. 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