ArticleProceedings of the National Academy of Sciences of the United States of America1998
A member of a family of sulfate-activating enzymes causes murine brachymorphism.
Article in Proceedings of the National Academy of Sciences of the United States of America, 1998. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 42 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
42 citing papers in PubMed, 152 citations in OpenAlex.
- MESH1 functions as a metazoan PAPS phosphatase to regulate sulfation.Nature chemical biology · 2026Article
- MESH1 regulates sulfation through the hydrolysis of PAPS.Nature chemical biology · 2026Article
- Article
- Correlation of 3'-phosphoadenosine-5'-phosphosulfate synthase 1 (PAPSS1) expression with clinical parameters and prognosis in esophageal squamous cell carcinoma.Histology and histopathology · 2023Article
- Chemistry and Function of Glycosaminoglycans in the Nervous System.Advances in neurobiology · 2023Review
- Disease-Related Protein Variants of the Highly Conserved Enzyme PAPSS2 Show Marginal Stability and Aggregation in Cells.Frontiers in molecular biosciences · 2022Article
- Roles of Chondroitin Sulfate Proteoglycans as Regulators of Skeletal Development.Frontiers in cell and developmental biology · 2022Review
- Congenital Disorders of Deficiency in Glycosaminoglycan Biosynthesis.Frontiers in genetics · 2021Review
- Cellular ATP Levels Determine the Stability of a Nucleotide Kinase.Frontiers in molecular biosciences · 2021Article
- Review
- Skeletal Dysplasias Caused by Sulfation Defects.International journal of molecular sciences · 2020Review
- Heparan Sulfate Proteoglycan Clustering in Wnt Signaling and Dispersal.Frontiers in cell and developmental biology · 2020Review
- Comparative analysis demonstrates cell type-specific conservation of SOX9 targets between mouse and chicken.Scientific reports · 2019Article
- Melting Down Protein Stability: PAPS Synthase 2 in Patients and in a Cellular Environment.Frontiers in molecular biosciences · 2019Review
- Sex-specific effects of serum sulfate level andMolecular genetics and metabolism reports · 2017Article
- The Regulation of Steroid Action by Sulfation and Desulfation.Endocrine reviews · 2015Review
- Sulphate in pregnancy.Nutrients · 2015Review
- Crystal structures of the kinase domain of the sulfate-activating complex in Mycobacterium tuberculosis.PloS one · 2015Article
- Article
- Human genetic disorders and knockout mice deficient in glycosaminoglycan.BioMed research international · 2014Review
Corrections and comments
- Erratum issued
Authors and funding
7 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Sulfation is critical to the function of a wide variety of biomolecules. This common modification requires the enzymatic synthesis of an activated sulfate donor, phosphoadenosine-phosphosulfate (PAPS). In higher organisms PAPS synthesis is catalyzed by a bifunctional sulfurylase kinase (SK) polypeptide having both ATP-sulfurylase and adenosine-phosphosulfate kinase activities. We report the identification of a gene family encoding murine SK proteins with these two activities. A family member, SK2, colocalizes with the locus for the autosomal recessive murine phenotype brachymorphism. Brachymorphic mice have normal lifespans, but abnormal hepatic detoxification, bleeding times, and postnatal growth, the latter being attributed to undersulfation of cartilage proteoglycan. A missense mutation in the SK2 coding sequence of bm mice that alters a highly conserved amino acid residue destroys adenosine-phosphosulfate kinase activity and therefore the ability of SK2 to synthesize PAPS. We conclude that a family of SK genes are responsible for sulfate activation in mammals, that a mutation in SK2 causes murine brachymorphism, and that members of this gene family have nonredundant, tissue-specific roles.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.