GATD3
GATD3 (glutamine amidotransferase class 1 domain containing 3) is a mitochondrial matrix protein that acts as a deglycase, removing early glycation adducts from amino acids and nucleotides. It is also known as KNP-Ia, ES1, HES1, GT335 and C21orf33. Browse Assay Genie's current range of GATD3 research reagents below, or read on for the biology behind the target.
GATD3 acts as a mitochondrial deglycase. It is involved in removing early non-enzymatic glycation adducts from amino acids and nucleotides, which restricts the formation of advanced glycation end products (AGEs) within mitochondria. By keeping glycation low on mitochondrial ribosomal RNA and proteins, GATD3 supports efficient mitochondrial gene expression. It also contributes to mitochondrial homeostasis, normal respiratory capacity and proper organisation of the mitochondrial network.
GATD3 has a second role as a regulator of malate dehydrogenase MDH2. It competitively limits the association of MDH2 with the mitochondrial deacetylase SIRT3, which preserves MDH2 acetylation and enzymatic activity. Through this mechanism it supports the conversion of malate to oxaloacetate, sustaining TCA cycle continuity and oxidative phosphorylation. The protein is localised to the mitochondrial matrix and belongs to the GATD3 family.
The human GATD3 gene encodes a potential mitochondrial protein that NCBI Gene places in the DJ-1/PfpI gene family, and alternate splicing gives multiple transcript variants. The protein is overexpressed in foetal Down syndrome brain. Earlier names reflect its history: chromosome 21 open reading frame 33 (C21orf33) and glutamine amidotransferase like class 1 domain containing 3A (GATD3A), with further aliases including KNP-I, KNPH, ES1 and HES1. A mouse ortholog, Gatd3, is recorded.
GATD3 removes early glycation adducts from amino acids and nucleotides, limiting AGE formation inside mitochondria.
It keeps glycation low on mitochondrial ribosomal RNA and proteins, supporting efficient mitochondrial gene expression.
By limiting MDH2 association with SIRT3, GATD3 preserves MDH2 activity and sustains TCA cycle continuity and oxidative phosphorylation.
GATD3 is overexpressed in foetal Down syndrome brain and is encoded on chromosome 21, making it relevant to studies of the condition.
GATD3 is a mitochondrial deglycase that removes early glycation adducts from amino acids and nucleotides, restricting AGE formation. It maintains low glycation on mitochondrial rRNA and proteins, and regulates MDH2 by limiting its association with SIRT3, supporting the TCA cycle and oxidative phosphorylation.
GATD3 has been called KNP-Ia, KNP-I, KNPI, KNPH, GT335, ES1, HES1, D21S2048E, C21orf33 and GATD3A. Previous names include chromosome 21 open reading frame 33 and glutamine amidotransferase like class 1 domain containing 3A. Alternate splicing produces multiple transcript variants.
NCBI Gene reports that GATD3 is overexpressed in foetal Down syndrome brain. The protein belongs to the DJ-1/PfpI gene family, and the mouse ortholog is Gatd3. Check the datasheet of any product for its validated reactivity.
Mitochondrial regulators, TCA cycle enzymes and DJ-1/PfpI family proteins with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt P0DPI2; product specifications from Assay Genie datasheets.