# PCMTD1

Source: https://onco.cc/targets/pcmtd1/  
OnCo record `pcmtd1` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

PCMTD1 (Protein-L-isoaspartate O-methyltransferase domain-containing protein 1) is a gene whose normal job is to hold cell growth in check. The public catalogues list it as a tumour suppressor, and it is called a cancer driver by mutation analysis of patient cohorts. Tied to Acute myeloid leukaemia.

## Summary

Substrate recognition component of an ECS (Elongin BC-CUL5-SOCS-box protein) E3 ubiquitin ligase complex which mediates the ubiquitination and subsequent proteasomal degradation of target proteins. Specifically binds to the methyltransferase cofactor S-adenosylmethionine (AdoMet) via the N-terminal AdoMet binding motif, but does not display methyltransferase activity. May provide an alternate maintenance pathway for modified proteins by acting as a damage-specific E3 ubiquitin ligase adaptor protein.

IntOGen calls it a driver in 1 cohort (0 activating, 1 loss-of-function), covering Acute Myeloid Leukaemia.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: protein-L-isoaspartate (D-aspartate) O-methyltransferase domain containing 1; Protein-L-isoaspartate O-methyltransferase domain-containing protein 1; FLJ10883
- Tags: cancer-genes-wave
- Symbol: PCMTD1
- Class: tumor-suppressor
- Biology: Substrate recognition component of an ECS (Elongin BC-CUL5-SOCS-box protein) E3 ubiquitin ligase complex which mediates the ubiquitination and subsequent proteasomal degradation of target proteins. Specifically binds to the methyltransferase cofactor S-adenosylmethionine (AdoMet) via the N-terminal AdoMet binding motif, but does not display methyltransferase activity. May provide an alternate maintenance pathway for modified proteins by acting as a damage-specific E3 ubiquitin ligase adaptor protein. Location: Cytoplasm; Membrane (UniProt). Locus 8q11.23 (HGNC).
- Where found: Acute myeloid leukaemia: IntOGen driver in 1 cohort (AML)

## Notes

- Written by scripts/fetch-cancer-genes.ts from CIViC, Open Targets, IntOGen, HGNC and UniProt; the function text is UniProt's, condensed and in UK spelling. Roles: IntOGen calls it a loss-of-function (LoF) driver in 1 cohort. Evidence tier "cohort-driver" is the strongest of those signals.
- Prevalence not recorded: none of the sources gives a positivity rate.

## Sources

- HGNC HGNC:30483: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:30483
- UniProt Q96MG8: https://www.uniprot.org/uniprotkb/Q96MG8/entry
- NCBI Gene 115294: https://www.ncbi.nlm.nih.gov/gene/115294
- Ensembl ENSG00000168300: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000168300

## Connected records

- collections: [IntOGen](https://onco.cc/collections/intogen/)
- cancers: [Acute myeloid leukaemia](https://onco.cc/cancers/aml/)

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JSON: https://onco.cc/api/v1/entities/pcmtd1.json