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3 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ZNG1B
Zn regulated GTPase metalloprotein activator 1B
Chromosome 2 · 2q14.1
NCBI Gene: 150472Ensembl: ENSG00000136682.16HGNC: HGNC:17907UniProt: C9J3G2
27PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
zinc ion bindingzinc chaperone activityprotein maturationcytoplasminsomniadisease of peritoneumcongenital hydronephrosisureter cancer
✦AI Summary

ZNG1B is a zinc chaperone that functions as a GTP-dependent zinc transferase, directly transferring zinc cofactors to activate target metalloproteins 1. Structurally, ZNG1B contains an N-terminal psi-PxLVp motif and a CXCC motif within its GTPase domain that mediates zinc binding and transfer. Its primary substrate is methionine aminopeptidase 1 (METAP1), an essential protein that cleaves initiator methionines during protein maturation. ZNG1B catalyzes zinc insertion into METAP1's active site through a mechanism requiring GTP hydrolysis and subsequent GTP/GDP exchange for release of the activated enzyme 1. Deletion of ZNG1 in yeast results in defective METAP1 function and zinc-deficiency growth defects, demonstrating the physiological importance of zinc transfer 1. ZNG1B operates in both cytoplasmic and nuclear compartments, positioning it to support zinc-dependent protein maturation across cellular environments. While the provided abstracts do not directly establish ZNG1B mutations in human disease, the functional dependence on ZNG1 for METAP1 activation and the broader relevance of zinc chaperones in human development suggest potential relevance to metal homeostasis-dependent pathologies, though specific clinical significance remains to be determined.

Sources cited
1
ZNG1B is a GTP-dependent zinc transferase that transfers zinc to methionine aminopeptidase, with deletion causing defective MetAP1 function and zinc-deficiency growth defects
PMID: 35584675
2
ZNG1B is located in the chromosome 2 fusion region with functional relevance to human biology through gene duplication and rearrangement processes
PMID: 12421752
⚠Limited data available — This gene has 2 indexed publications. Summary and analysis may be incomplete.
Disease Associationsⓘ20
insomniaOpen Targets
0.11Weak
disease of peritoneumOpen Targets
0.10Weak
congenital hydronephrosisOpen Targets
0.07Suggestive
ureter cancerOpen Targets
0.07Suggestive
ureteral neoplasmOpen Targets
0.07Suggestive
ureteroceleOpen Targets
0.07Suggestive
Genetic renal or urinary tract malformationOpen Targets
0.07Suggestive
familial vesicoureteral refluxOpen Targets
0.07Suggestive
congenital primary megaureterOpen Targets
0.06Suggestive
Ochoa syndromeOpen Targets
0.06Suggestive
vesicoureteral reflux 3Open Targets
0.06Suggestive
renal hypodysplasia/aplasia 3Open Targets
0.06Suggestive
congenital anomalies of kidney and urinary tract 1Open Targets
0.05Suggestive
caudal duplicationOpen Targets
0.05Suggestive
renal dysplasiaOpen Targets
0.05Suggestive
primary hyperoxaluria type 2Open Targets
0.05Suggestive
urofacial syndrome 2Open Targets
0.05Suggestive
renal hypoplasiaOpen Targets
0.05Suggestive
thymic-renal-anal-lung dysplasiaOpen Targets
0.05Suggestive
circadian rhythmOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
NAA10Shared pathway100%YAE1Shared pathway100%NAA16Shared pathway100%NAA15Shared pathway100%LMF2Shared pathway100%CHCHD4Shared pathway100%
Tissue Expression6 tissues
Bone Marrow
100%
Ovary
85%
Brain
81%
Lung
79%
Liver
77%
Heart
77%
Gene Interaction Network
Click a node to explore
ZNG1BNAA10YAE1NAA16NAA15LMF2CHCHD4
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q8IUF1
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.57Moderately Constrained
pLIⓘ
0.86Intermediate
Observed/Expected LoF0.34 [0.21–0.57]
RankingsWhere ZNG1B stands among ~20K protein-coding genes
  • #12,721of 20,598
    Most Researched27
  • #3,777of 17,882
    Most Constrained (LOEUF)0.57 · top quartile
Genes detectedZNG1B
Sources retrieved3 papers
Response time—
📄 Sources
3
1
Gene content and function of the ancestral chromosome fusion site in human chromosome 2q13-2q14.1 and paralogous regions.
PMID: 12421752
Genome Res · 2002
1.00
2
Zng1 is a GTP-dependent zinc transferase needed for activation of methionine aminopeptidase.
PMID: 35584675
Cell Rep · 2022
0.67
3
Deletion in the
PMID: 31862704
J Am Soc Nephrol · 2020
0.33