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Overview

Uniprot ID	Q925I1
Protein Name	ATPase family AAA domain-containing protein 3A
Gene Name	Atad3a
Organism	Mus musculus

Kla Sites from experimental identification

Position	Flanking peptide
423	LRKRATEKISEDLRA
500	LKPATEGKQRLKVAQ
504	TEGKQRLKVAQFDYG

Function

Essential for mitochondrial network organization, mitochondrial metabolism and cell growth at organism and cellular level. May play an important role in mitochondrial protein synthesis. May also participate in mitochondrial DNA replication. May bind to mitochondrial DNA D-loops and contribute to nucleoid stability. Required for enhanced channeling of cholesterol for hormone-dependent steroidogenesis. Involved in mitochondrial-mediated antiviral innate immunity. Required to protect mitochondria from the PERK-mediated unfolded protein response: specifically inhibits the activity of EIF2AK3/PERK at mitochondria-endoplasmic reticulum contact sites, thereby providing a safe haven for mitochondrial protein translation during endoplasmic reticulum stress. Ability to inhibit EIF2AK3/PERK is independent of its ATPase activity. Also involved in the mitochondrial DNA damage response by promoting signaling between damaged genomes and the mitochondrial membrane, leading to activation of the integrated stress response (ISR)

Protein Sequence

10 MSWLFGIKGP 20 KGEGTGPPLP 30 LPPAQPGAEG 40 GGDRGAGDRP 50 SPKDKWSNFD 60 PTGLERAAKA 70 ARELEHSRHA 80 KEALSLAQMQ 90 EQTLQLEQQS 100 KLKEYEAAVE 110 QLKSEQIRVQ 120 AEERRKTLTE 130 ETRQHQARAQ 140 YQDKLARQRY 150 EDQLKQQQLL 160 NEENLRKQEE 170 SVQKQEAIRR 180 ATVEREMELR 190 HKNEMLRVEA 200 EARARAKADR 210 ENADIIREQI 220 RLKAAEHRQT 230 ILESIRTAGT 240 LLGEGFRAFV 250 TDWDKVTATV 260 AGLTLLAVGV 270 YSAKNATSVA 280 GRYIEARLGK 290 PSLVRETSRI 300 SVLEALRHPI 310 QVSRRLVSRP 320 QDALEGVILS 330 PSLEARVRDI 340 AIATRNTKKN 350 KSLYRNVLMY 360 GPPGTGKTLF 370 AKKLALHSGM 380 DYAIMTGGDV 390 APMGREGVTA 400 MHKVFDWAST 410 SRRGLLLFVD 420 EADAFLRKRA 430 TEKISEDLRA 440 TLNAFLHRTG 450 QHSSKFMLVL 460 ASNQPEQFDW 470 AINDRIDEMV 480 CFALPQREER 490 ERLVRMYFDK 500 YVLKPATEGK 510 QRLKVAQFDY 520 GKKCSEVAQL 530 TEGMSGREIA 540 QLAVAWQAMA 550 YSSEDGVLTE 560 AMMDARVQDA 570 VQQHQQKMQW 580 LKVERPDSQT 590 NKPPHPSLLS C

Gene Ontology

Classification	GO ID	Description
Cellular Component	GO:0044233	mitochondria-associated endoplasmic reticulum membrane contact site
Cellular Component	GO:0005743	mitochondrial inner membrane
Cellular Component	GO:0042645	mitochondrial nucleoid
Cellular Component	GO:0005739	mitochondrion
Molecular Function	GO:0005524	ATP binding
Molecular Function	GO:0016887	ATP hydrolysis activity
Molecular Function	GO:0042802	identical protein binding
Molecular Function	GO:0030291	protein serine/threonine kinase inhibitor activity
Biological Process	GO:0140374	antiviral innate immune response
Biological Process	GO:0006974	DNA damage response
Biological Process	GO:0140468	HRI-mediated signaling
Biological Process	GO:0007005	mitochondrion organization
Biological Process	GO:0043066	negative regulation of apoptotic process
Biological Process	GO:1903898	negative regulation of PERK-mediated unfolded protein response
Biological Process	GO:0001558	regulation of cell growth

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.