Search Results

Overview

Uniprot IDQ9CZN7
Protein NameSerine hydroxymethyltransferase, mitochondrial
Gene NameShmt2
OrganismMus musculus

Kla Sites from experimental identification

Position Flanking peptide
103 YSEGYPGKRYYGGAE
367 AMADALLKRGYSLVS
464 EVKRKTAKLQDFKSF
474 DFKSFLLKDPETSQR

Function

Catalyzes the cleavage of serine to glycine accompanied with the production of 5,10-methylenetetrahydrofolate, an essential intermediate for purine biosynthesis (By similarity). Serine provides the major source of folate one-carbon in cells by catalyzing the transfer of one carbon from serine to tetrahydrofolate (By similarity). Contributes to the de novo mitochondrial thymidylate biosynthesis pathway via its role in glycine and tetrahydrofolate metabolism: thymidylate biosynthesis is required to prevent uracil accumulation in mtDNA (By similarity). Also required for mitochondrial translation by producing 5,10-methylenetetrahydrofolate; 5,10-methylenetetrahydrofolate providing methyl donors to produce the taurinomethyluridine base at the wobble position of some mitochondrial tRNAs (PubMed:29452640). Associates with mitochondrial DNA (By similarity). In addition to its role in mitochondria, also plays a role in the deubiquitination of target proteins as component of the BRISC complex: required for IFNAR1 deubiquitination by the BRISC complex (By similarity)

Protein Sequence

10 MVSFSLLRTT 20 RPLQRCGQLV 30 CMAARAQHSK 40 VAQTQAGEAA 50 GGWTGQESLS 60 DSDPEMWELL 70 QREKDRQCRG 80 LELIASENFC 90 SRAALEALGS 100 CLNNKYSEGY 110 PGKRYYGGAE 120 VVDEIELLCQ 130 RRALEAFDLD 140 PAQWGVNVQP 150 YSGSPANLAA 160 YTALLQPHDR 170 IMGLDLPDGG 180 HLTHGYMSDV 190 KRISATSIFF 200 ESMPYKLNPQ 210 TGLIDYDQLA 220 LTARLFRPRL 230 IIAGTSAYAR 240 LIDYARMREV 250 CDEVRAHLLA 260 DMAHISGLVA 270 AKVIPSPFKY 280 ADVVTTTTHK 290 TLRGARSGLI 300 FYRKGVRTVD 310 PKTGKEIPYT 320 FEDRINFAVF 330 PSLQGGPHNH 340 AIAAVAVALK 350 QACTPMFREY 360 SLQVLRNAQA 370 MADALLKRGY 380 SLVSGGTDTH 390 LVLVDLRPKG 400 LDGARAERVL 410 ELVSITANKN 420 TCPGDRSAIT 430 PGGLRLGAPA 440 LTSRQFREDD 450 FRRVVDFIDE 460 GVNIGLEVKR 470 KTAKLQDFKS 480 FLLKDPETSQ 490 RLANLRQQVE 500 QFARGFPMPG FDER

Gene Ontology

Classification GO ID Description
Cellular Component GO:0070552 BRISC complex
Cellular Component GO:0005737 cytoplasm
Cellular Component GO:0015630 microtubule cytoskeleton
Cellular Component GO:0005743 mitochondrial inner membrane
Cellular Component GO:0005759 mitochondrial matrix
Cellular Component GO:0042645 mitochondrial nucleoid
Cellular Component GO:0005739 mitochondrion
Cellular Component GO:0005634 nucleus
Molecular Function GO:0016597 amino acid binding
Molecular Function GO:0003682 chromatin binding
Molecular Function GO:0004372 glycine hydroxymethyltransferase activity
Molecular Function GO:0120567 hydroxytrimethyllysine aldolase activity
Molecular Function GO:0042802 identical protein binding
Molecular Function GO:0008732 L-allo-threonine aldolase activity
Molecular Function GO:0030170 pyridoxal phosphate binding
Biological Process GO:0015943 formate biosynthetic process
Biological Process GO:0006545 glycine biosynthetic process
Biological Process GO:0019264 glycine biosynthetic process from L-serine
Biological Process GO:0006544 glycine metabolic process
Biological Process GO:0006564 L-serine biosynthetic process
Biological Process GO:0006563 L-serine metabolic process
Biological Process GO:0006730 one-carbon metabolic process
Biological Process GO:0008284 positive regulation of cell population proliferation
Biological Process GO:0051289 protein homotetramerization
Biological Process GO:0070536 protein K63-linked deubiquitination
Biological Process GO:0051262 protein tetramerization
Biological Process GO:1903715 regulation of aerobic respiration
Biological Process GO:0070129 regulation of mitochondrial translation
Biological Process GO:0002082 regulation of oxidative phosphorylation
Biological Process GO:0034340 response to type I interferon
Biological Process GO:0035999 tetrahydrofolate interconversion
Biological Process GO:0046653 tetrahydrofolate metabolic process

Reference

[1] Zhuo W, Zhang M, Tan J, Gao Y, Wang Y et al.. Lysine lactylation analysis of proteins in the heart of the Kawasaki disease mouse model.. Front Cell Dev Biol 13:1550220. 2025. PMID: 40114965.

[2] Wu D, Tang Y, Li X, Xiong S, Zhang Z et al.. Characterization of protein lactylation in healthy and ischemic mouse hearts.. Front Cardiovasc Med 12:1644886. 2025. PMID: 41089239.