Search Results

Overview

Uniprot ID	Q9D172
Protein Name	Glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
Gene Name	Gatd3
Organism	Mus musculus

Kla Sites from experimental identification

Position	Flanking peptide
149	STFAVDGKDCKVNKE
155	GKDCKVNKEVERVLK
162	KEVERVLKEFHGAKK
168	LKEFHGAKKPIGLCC
186	VLAAKVIKGVEVTVG
201	HEQEEGGKWPYAGTA
217	AIKALGAKHCVKGVT
221	LGAKHCVKGVTEAHV
231	TEAHVDQKNKVVTTP

Function

Acts as a mitochondrial deglycase involved in the removal of early non-enzymatic glycation adducts from amino acids and nucleotides, thereby restricting the formation of advanced glycation end products (AGEs) within mitochondria. Required to maintain low levels of glycation on mitochondrial ribosomal RNA and proteins, supporting efficient mitochondrial gene expression. Contributes to mitochondrial homeostasis, normal respiratory capacity, and proper mitochondrial network organization. Acts also as a mitochondrial regulator of malate dehydrogenase MDH2 by competitively limiting the association of MDH2 with the mitochondrial deacetylase SIRT3, thereby preserving MDH2 acetylation and enzymatic activity. Through this mechanism, supports efficient conversion of malate to oxaloacetate, sustaining TCA cycle continuity and oxidative phosphorylation

Protein Sequence

10 MAAVRVLVAP 20 RLASALLPLS 30 RYHRAPSQRA 40 ALHSSAPRPG 50 ARVALVLSGC 60 GVYDGTEIHE 70 ASAILVHLSR 80 GGAEVQIFAP 90 DVPQMHVIDH 100 TKGEPSERES 110 RNVLAESARI 120 ARGKITSLAQ 130 LNAANHDAAI 140 FPGGFGAAKN 150 LSTFAVDGKD 160 CKVNKEVERV 170 LKEFHGAKKP 180 IGLCCIAPVL 190 AAKVIKGVEV 200 TVGHEQEEGG 210 KWPYAGTAEA 220 IKALGAKHCV 230 KGVTEAHVDQ 240 KNKVVTTPAF 250 MCETALHHIH 260 DGIGAMVKNV LELTGK

Gene Ontology

Classification	GO ID	Description
Cellular Component	GO:0005739	mitochondrion

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.