Search Results

Overview

Uniprot ID	P0DPI2
Protein Name	Glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
Gene Name	GATD3
Organism	Homo sapiens

Kla Sites from experimental identification

Position	Flanking peptide
116	SARIARGKITDLANL
141	PGGFGAAKNLSTFAV
151	STFAVDGKDCKVNKE
157	GKDCKVNKEVERVLK
164	KEVERVLKEFHQAGK
203	HEQEEGGKWPYAGTA
219	AIKALGAKHCVKEVV
223	LGAKHCVKEVVEAHV
233	VEAHVDQKNKVVTTP
261	GIGAMVRKVLELTGK
94	MHVIDHTKGQPSEGE

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 (PubMed:35307029). 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 (PubMed:39738099)

Protein Sequence

10 MAAVRVLVAS 20 RLAAASAFTS 30 LSPGGRTPSQ 40 RAALHLSVPR 50 PAARVALVLS 60 GCGVYDGTEI 70 HEASAILVHL 80 SRGGAEVQIF 90 APDVPQMHVI 100 DHTKGQPSEG 110 ESRNVLTESA 120 RIARGKITDL 130 ANLSAANHDA 140 AIFPGGFGAA 150 KNLSTFAVDG 160 KDCKVNKEVE 170 RVLKEFHQAG 180 KPIGLCCIAP 190 VLAAKVLRGV 200 EVTVGHEQEE 210 GGKWPYAGTA 220 EAIKALGAKH 230 CVKEVVEAHV 240 DQKNKVVTTP 250 AFMCETALHY 260 IHDGIGAMVR KVLELTGK

Gene Ontology

Classification	GO ID	Description
Cellular Component	GO:0005739	mitochondrion

Reference

[1] Yang Z, Yan C, Ma J, Peng P, Ren X et al.. Lactylome analysis suggests lactylation-dependent mechanisms of metabolic adaptation in hepatocellular carcinoma.. Nat Metab 5(1):61-79. 2023 Jan. PMID: 36593272.

[2] Hong H, Chen X, Wang H, Gu X, Yuan Y et al.. Global profiling of protein lysine lactylation and potential target modified protein analysis in hepatocellular carcinoma.. Proteomics 23(9):e2200432. 2023 May. PMID: 36625413.

[3] Shi CM, Wang QC, Li XL, Yang YH, Tang XY et al.. Global Profiling of Protein Lactylation in Human Hippocampi.. Proteomics Clin Appl 19(2):e202400061. 2025 Mar. PMID: 39610256.