GATD3
entrez
8209
canonical
uniprot
P0DPI2
canonical
ensembl
ENSG00000160221
resolver
ensp
ENSP00000291577
resolver
ensp
ENSP00000344901
resolver
ensp
ENSP00000374340
resolver
ensp
ENSP00000396655
resolver
ensp
ENSP00000405713
resolver
enst
ENST00000427803
resolver
genesymbol
GATD3
resolver
genesymbol_syn
C21orf33
resolver
genesymbol_syn
GATD3A
resolver
hgnc
HGNC:1273
resolver
uniprot
A0A140VKB0
resolver
uniprot
A6NFJ6
resolver
uniprot
A6NJY7
resolver
uniprot
F2Z2Q0
resolver
uniprot
O00650
resolver
uniprot
O00660
resolver
uniprot
O15011
resolver
uniprot
O15012
resolver
uniprot
P30042
resolver
uniprot
P55346
resolver
uniprot
P78474
resolver
uniprot
Q92505
resolver
uniprot
Q92507
resolver
uniprot_entry
A0A140VKB0_HUMAN
resolver
uniprot_entry
F2Z2Q0_HUMAN
resolver
uniprot_entry
GAL3A_HUMAN
resolver
uniprot_sec
A6NFJ6
resolver
uniprot_sec
A6NJY7
resolver
uniprot_sec
O00650
resolver
uniprot_sec
O00660
resolver
uniprot_sec
O15011
resolver
uniprot_sec
O15012
resolver
uniprot_sec
P30042
resolver
uniprot_sec
P55346
resolver
uniprot_sec
P78474
resolver
uniprot_sec
Q92505
resolver
uniprot_sec
Q92507
resolver
amino_acid_sequence
MAAVRVLVASRLAAASAFTSLSPGGRTPSQRAALHLSVPRPAARVALVLSGCGVYDGTEIHEASAILVHLSRGGAEVQIFAPDVPQMHVIDHTKGQPSEGESRNVLTESARIARGKITDLANLSAANHDAAIFPGGFGAAKNLSTFAVDGKDCKVNKEVERVLKEFHQAGKPIGLCCIAPVLAAKVLRGVEVTVGHEQEEGGKWPYAGTAEAIKALGAKHCVKEVVEAHVDQKNKVVTTPAFMCETALHYIHDGIGAMVRKVLELTGK
uniprot
proteins
function
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). {ECO:0000269|PubMed:35307029, ECO:0000269|PubMed:39738099}.
uniprot
proteins
mass_dalton
28170
uniprot
proteins
mutagenesis
MUTAGEN 176; /note="C->A: Strongly diminished amidolytic activity."; /evidence="ECO:0000269|PubMed:35307029
uniprot
proteins
protein_family
GATD3 family
uniprot
proteins
pubmed
10830953
uniprot
proteins
pubmed
1286669
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
35307029
uniprot
proteins
pubmed
39738099
uniprot
proteins
pubmed
9205129
uniprot
proteins
sequence_length
268
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion matrix {ECO:0000269|PubMed:35307029}.
uniprot
proteins