GATD3A Glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial P0DPI2 GAL3A_HUMAN
Swiss-Prot reviewed
Mutations
348
CL 23 · Tissue 315
Samples
119
CL 11 · Tissue 105
Peptides
117
unique mutant peptides
Transcripts
4
isoforms mutated

Stats by Source

Global, split by cell line (COSMIC CL / DepMap / PubMed) vs tissue (COSMIC primary tissue)

How the counts split by source
Stats by Source

Global = all mutations for this gene across every source.

Cell line = COSMIC Cell Lines Project + DepMap + PubMed.

Tissue = COSMIC primary-tissue (patient tumour) samples.

Global can exceed cell line + tissue: COSMIC tissue-derived models sit only in global, and a peptide can be shared across both.

GlobalCell lineTissue
Mutations34823315
Samples11911105
Peptides11713106

Function

GATD3A · Glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial

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)

Isoforms & Proteins

4 transcripts · UniProt mapping is sequence-verified (AA-safe)

About the isoform mapping
Isoforms & Proteins

Each Ensembl transcript (ENST) this gene is mutated on, with its matched UniProt accession.

The mapping is sequence-verified: the UniProt sequence is identical to the transcript translation, so amino-acid positions line up exactly. A * marks an unreviewed (TrEMBL) entry.

Counts are mutations and unique mutant peptides on each transcript.

TranscriptUniProtMutationsPeptides
ENST00000291577 P0DPI2 115 92
ENST00000348499 P0DPI2-2 103 82
ENST00000427803 F2Z2Q0* 73 58
ENST00000644251 A0A2R8Y6K9* 57 44

Gene Properties

Recurrent Mutations

Top recurrent amino-acid changes along the protein · needle height = number of mutations

What this lollipop shows
Recurrent Mutations

A lollipop / needle plot – the standard way to show recurrent mutations along a protein (as used by cBioPortal and MutationMapper).

X-axis = amino-acid position in the protein.

Needle height & head size = how often that exact amino-acid change was observed (its recurrence). Tall/large heads are mutational hotspots.

The most recurrent changes are labelled; hover any needle for the change, position and counts.

Mutation Distribution

Where GATD3A is mutated · all tissues, split by cell line vs tissue

Mutation counts by tissue
Mutation Distribution

How many mutations in GATD3A were found in each tissue, across the whole database.

Each bar is a tissue (cell-line and tissue names are merged to the standard tissue), split into cell line and tissue (patient tumour) contributions.

This shows the cancer-context where this gene is recurrently altered.

Mutations

All 348 mutations in GATD3A

About the mutation list
Mutations

Every mutation record for this gene, across all samples and sources.

The Sample column links to the cell line (cell-line samples) or the tissue type (tissue samples).

Use the Type / Source filters, the search box, and column sorting to explore; each CAN-IMMUNE ID opens the full mutation & peptide view.

IDSampleTranscriptAA Change CDSTypeSourcePeptide