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Overview

Uniprot IDP97807
Protein NameFumarate hydratase, mitochondrial
Gene NameFh
OrganismMus musculus

Kla Sites from experimental identification

Position Flanking peptide
112 QEYGLDPKIASAIMK
119 KIASAIMKAADEVAE
169 LGGELGSKKPVHPND
170 GGELGSKKPVHPNDH
218 LHDALSAKSKEFAQV
220 DALSAKSKEFAQVIK
260 QYAMVRIKAAMPRIY
289 TRIGFAEKVAAKVAA
501 FDEWVKPKDMLGPK*
58 FDTFGELKVPTDKYY
63 ELKVPTDKYYGAQTV
77 VRSTMNFKIGGATER

Function

Catalyzes the reversible stereospecific interconversion of fumarate to L-malate (PubMed:23643539). Experiments in different species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable)

Protein Sequence

10 MYRALRLLAR 20 SRRLLRVPSA 30 GAAVSGEATT 40 LPRCAPNVAR 50 MASQNSFRVE 60 FDTFGELKVP 70 TDKYYGAQTV 80 RSTMNFKIGG 90 ATERMPIPVI 100 QAFGILKRAA 110 AEVNQEYGLD 120 PKIASAIMKA 130 ADEVAEGKLN 140 DHFPLVVWQT 150 GSGTQTNMNV 160 NEVISNRAIE 170 MLGGELGSKK 180 PVHPNDHVNK 190 SQSSNDTFPT 200 AMHIAAAVEV 210 HKVLLPGLQK 220 LHDALSAKSK 230 EFAQVIKIGR 240 THTQDAVPLT 250 LGQEFSGYVQ 260 QVQYAMVRIK 270 AAMPRIYELA 280 AGGTAVGTGL 290 NTRIGFAEKV 300 AAKVAALTGL 310 PFVTAPNKFE 320 ALAAHDALVE 330 LSGAMNTAAC 340 SLMKIANDIR 350 FLGSGPRSGL 360 GELILPENEP 370 GSSIMPGKVN 380 PTQCEAMTMV 390 AAQVMGNHVA 400 VTVGGSNGHF 410 ELNVFKPMMI 420 KNVLHSARLL 430 GDASVSFTDN 440 CVVGIQANTE 450 RINKLMNESL 460 MLVTALNPHI 470 GYDKAAKIAK 480 TAHKNGSTLK 490 ETAIELGYLT 500 AEQFDEWVKP KDMLGPK

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005694 chromosome
Cellular Component GO:0005829 cytosol
Cellular Component GO:0005739 mitochondrion
Cellular Component GO:0005634 nucleus
Molecular Function GO:0004333 fumarate hydratase activity
Biological Process GO:0006525 arginine metabolic process
Biological Process GO:0006974 DNA damage response
Biological Process GO:0006281 DNA repair
Biological Process GO:0006106 fumarate metabolic process
Biological Process GO:0006108 malate metabolic process
Biological Process GO:0120162 positive regulation of cold-induced thermogenesis
Biological Process GO:2001034 positive regulation of double-strand break repair via nonhomologous end joining
Biological Process GO:0006099 tricarboxylic acid cycle
Biological Process GO:0000050 urea cycle

Reference

[1] Chang J, Wu W, Qian P, Lu Z, He X et al.. Multi-omics study on the effect of moderate-intensity exercise on protein lactylation in mouse muscle tissue.. Front Cell Dev Biol 12:1472338. 2024. PMID: 39935788.

[2] 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.

[3] 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.