Seroatlas · Human Serome Atlas

ZNHIT3

Zinc finger HIT domain-containing protein 3

Also known as: Hit1, TRIP3, ZNHI3_HUMAN

Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene

Protein identityUniProt · HPA

UniProt accession: Q15649
Gene: ZNHIT3
Ensembl: ENSG00000273611
Chromosome: 17
Canonical length: 155 aa
Protein class: Disease related genes, Human disease related genes, Predicted intracellular proteins
Subcellular location: Mitochondria

OverviewNCBI Gene

Predicted to enable nuclear thyroid hormone receptor binding activity. Predicted to be involved in box C/D snoRNP assembly; maturation of LSU-rRNA from tricistronic rRNA transcript (SSU-rRNA, 5.8S rRNA, LSU-rRNA); and snoRNA localization. Located in cytoplasm and nucleus. Implicated in PEHO syndrome. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

155 residues, UniProt reviewed canonical sequence.

>Q15649|ZNHIT3
     1  MASLKCSTVV CVICLEKPKY RCPACRVPYC SVVCFRKHKE QCNPETRPVE KKIRSALPTK
    61  TVKPVENKDD DDSIADFLNS DEEEDRVSLQ NLKNLGESAT LRSLLLNPHL RQLMVNLDQG
   121  EDKAKLMRAY MQEPLFVEFA DCCLGIVEPS QNEES

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against ZNHIT3 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability: Intracellular
Secreted: No
Transmembrane segments: 0
Mean surface accessibility (rSASA): 0.49
Highest tissue expression: 61 nTPM

Expression across tissuesHPA

Tissue

cerebral cortex: 61 nTPM
amygdala: 60 nTPM
skeletal muscle: 59 nTPM
basal ganglia: 52 nTPM
hippocampal formation: 51 nTPM
testis: 49 nTPM

Single-cell type

other brain neurons: 22 nCPM
oligodendrocytes: 22 nCPM
brain excitatory neurons: 20 nCPM
brain inhibitory neurons: 18 nCPM
bergmann glia: 16 nCPM
oligodendrocyte progenitor cells: 14 nCPM

Immune cell

naive CD4 T-cell: 34 nTPM
MAIT T-cell: 33 nTPM
naive CD8 T-cell: 28 nTPM
T-reg: 27 nTPM
memory CD4 T-cell: 27 nTPM
non-classical monocyte: 27 nTPM

Brain region

white matter: 17 nTPM
spinal cord: 17 nTPM
hypothalamus: 17 nTPM
cerebral cortex: 16 nTPM
basal ganglia: 15 nTPM
midbrain: 14 nTPM

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about ZNHIT3.

Disease | AllUniProt

Conditions ZNHIT3 is implicated in, by any mechanism.

PEHO syndrome (PEHO) MIM:260565

Disease | GeneticClinVar

3 pathogenic / likely-pathogenic of 57 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

PEHO syndrome

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance): 1.8
gnomAD pLI: 0
gnomAD missense Z: -0.6
DepMap mean gene effect: -0.53
DepMap dependency class: common

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 5% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

Molecular functions

Cellular components

Protein domainsUniProt · Pfam · InterPro

Zinc finger, HIT-type
Box C/D snoRNA
HIT zinc finger
Zinc finger HIT domain-containing protein 3, C-terminal domain
Zinc finger HIT domain-containing protein 3, C-terminal domain

KeywordsUniProt

InteractionsUniProt · HPA

Protein binding partners of ZNHIT3 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

NUFIP1

Antibody and autoantibody relevanceSeroatlas analysis

Seroatlas reads ZNHIT3 as an antibody target. Whether an autoantibody or antibody against ZNHIT3 could matter depends on whether native ZNHIT3 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.

ZNHIT3 is annotated as predominantly intracellular. Intracellular proteins are common autoantibody markers, becoming visible to the immune system after cell injury or altered processing, but are usually markers of disease rather than direct drivers.

Annotation status

The present source text does not explicitly label ZNHIT3 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.

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