ZNF205

Chr 16

zinc finger protein 205

Also known as: RhitH, ZNF210, Zfp13

NCBI Gene: 7755 ENSG00000122386 UniProt: O95201 OMIM: 603436

ZNF205 encodes a transcriptional repressor that regulates MPV17L expression and contributes to hydrogen peroxide metabolism and mitochondrial apoptotic pathways. The gene shows low constraint to loss-of-function variation (pLI = 0.03, LOEUF = 0.56), but no established human disease associations have been reported to date. Clinical significance of ZNF205 variants in pediatric neurological disorders remains to be determined.

OMIM ResearchSummary from RefSeq, UniProt

Predicted to enable DNA-binding transcription factor activity, RNA polymerase II-specific and RNA polymerase II cis-regulatory region sequence-specific DNA binding activity. Involved in negative regulation of transcription by RNA polymerase II; positive regulation of hydrogen peroxide biosynthetic process; and positive regulation of mitochondrial outer membrane permeabilization involved in apoptotic signaling pathway. Predicted to be located in mitochondrion and nucleus. [provided by Alliance of Genome Resources, Jul 2025]

OMIM ResearchGenerating clinical summary…

Clinical highlights

Interpreting a novel variant

This gene is strongly intolerant of loss-of-function variation in the population, so LoF variants warrant close attention. No curated mechanism annotation is available — see the mechanism card for the computational prediction and its caveats.Based on population constraint only.

Active trials

Pubs (1 yr)

—

P/LP submissions

—

P/LP missense

0.56

LOEUF

Multiple*

Mechanism· predicted